Recurrent pyogenic granuloma or Warner and Wilson-Jones syndrome

Here we report a case of pneumococcal meningitis with bilateral sensorineural hearing loss at the onset. The patient was a 60-year-old man who a few days before visiting our hospital experienced common cold-like symptoms, and then he suddenly developed bilateral hearing loss. Examination of the cerebrospinal fluid (CSF) on the day of admission revealed pleocytosis and his CSF culture demonstrated pneumococci. Otorhinolaryngological examinations disclosed bilateral severe sensorineural hearing loss due to cochlear impairment. Many cases of bacterial meningitis concomitant with hearing loss have been reported, but a case of meningitis starting with sudden hearing loss is rare.     

Endolymphatic sac obliteration for large vestibular aqueduct syndrome

The objective of this study was to investigate the effects of endolymphatic sac obliteration for stabilization of progressive hearing loss in patients with the large vestibular aqueduct syndrome. This was a retrospective case review conducted at a private neurootologic office in a metropolitan area. Seven ears in six patients were subjected to surgery for obliteration of the endolymphatic sac in an effort to stabilize progressive hearing loss associated with the large vestibular aqueduct syndrome. The study population was composed of four boys and two girls 4-17 years of age. The large vestibular aqueduct was unilateral in two patients and bilateral in four patients. All seven ears demonstrated progressive sensorineural hearing loss preoperatively. Surgical tissue obliteration was performed via a transmastoid approach in seven ears. The main outcome measure was comparison of pre- and postoperative hearing levels and stability. Magnetic resonance imaging also was performed in all cases at least 6 months postoperatively to determine patency of the endolymphatic sac and vestibular aqueduct. Six of seven ears maintained stable hearing during the follow-up period, which ranged from 6 months to 6 years (mean 3.2 years). One patient showed continued progression of hearing loss postoperatively. All seven ears demonstrated continued obliteration on postoperative imaging studies. Surgical obliteration of the endolymphatic sac may stabilize hearing in patients with the large vestibular aqueduct syndrome and progressive hearing loss. These results support the theory of pressure or fluid reflux into the labyrinth as a cause of progressive hearing loss in these patients.     

Immunological abnormalities in patients with etiology unknown sensorineural hearing loss

An immunological study was carried out in 50 patients with etiology unknown sensorineural hearing loss, including the following: 12 low tone deafness, 7 sudden deafness, 8 unilateral deafness, 7 idiopathic bilateral progressive sensorineural hearing loss and 14 other bilateral sensorineural hearing loss. Twenty-five out of 50 of the cases demonstrated immunological abnormalities. A full array immunological tests were performed with the following results. High immnunoglobulin titers showed in 18 out of 50 cases. Six cases were positive for antinuclear antibody. The anti-DNA antibody assay revealed high titers in 2 cases. Also, anti-rheumatoid factor antibody assay showed high titers in 6 cases while abnormal values of complements in serum were detected in 6 cases, though an immune complex was demonstrated in only one case. Also, it was noted that six patients with immunological abnormalities have autoimmune diseases. The above results indicated that some cases of etiology unknown sensorineural hearing loss may have an immunological link.     

Evaluation of an automated auditory brainstem response infant hearing screening method in at risk neonates

An automated auditory brainstem response (ABR) method-the ALGO-1 Plus- has been developed for hearing screening in healthy neonates. The aim of this study was to test the validity of this automated ABR screening method in at-risk neonates in a neonatal intensive care unit. Two hundred and fifty at-risk neonates were selected for screening according to the criteria of the American Joint Commerce on Infant Hearing. All 250 neonates were screened with the ALGO-1 Plus for bilateral hearing loss. When two consecutive screenings pointed to bilateral hearing loss ("refer") further audiological investigations were performed and where necessary therapeutic measures were taken. All children who "passed" the screening unilateral or bilateral enrolled in a nationwide behavioural screening programme at the age of 9 months as well as in a 6-monthly follow up programme documenting speech and language development. A total of 245 (98%) neonates passed the ALGO-1 screening, 230 (92%) at the first attempt and 15 (6%) at the second attempt. Five (2%) were referred with bilateral hearing loss. One of these died of congenital rubella shortly after screening and bilateral congenital hearing loss of > 35 dB was confirmed in the other 4. None of the infants who passed the screening were discovered to have moderate to severe bilateral hearing loss (> 40 dB) with behavioural screening (n = 183/233) or at follow up (n = 233/233). In this study, all at-risk neonates with bilateral congenital hearing loss were detected with ALGO-1 Plus screening. No false-negatives were discovered. CONCLUSION: The ALGO-1 Plus infant hearing screener can be used as a valid automated ABR-screener to detect hearing loss in at-risk neonates in a neonatal intensive care setting.     

Auditory filter shapes of normal-hearing and hearing-impaired listeners in continuous broadband noise

Listeners with sensorineural hearing impairment typically exhibit auditory processing deficits such as reduced frequency and/or temporal resolution. Such deficits may represent separate sequela of auditory pathology or may result directly from the sensitivity loss and the requirement to listen at high levels. To assess the impact of increased thresholds on frequency resolution, auditory filter characteristics were determined for hearing-impaired and normal-hearing listeners at 500 and 2000 Hz in the presence of continuous broadband noise meant as a rough simulation of hearing loss. In the fitting procedure, the low-frequency skirt of the derived auditory filter was allowed to vary as a function of signal level, permitting different filter shapes to be estimated at high versus low signal levels. Listeners with moderate hearing losses at 2000 Hz demonstrated near-normal auditory filter shapes for lower signal levels, but increasingly broad and asymmetric filters as signal level was raised. At 500 Hz, where hearing losses were mild, filter bandwidths increased little at the higher signal levels. The presence of broadband noise had essentially no effect on filter shapes of either listener group. The filter shape abnormalities demonstrated by listeners with moderate hearing loss, which were not observed in normal-hearing listeners at the same signal levels, indicate that poor frequency resolution in these patients for high-intensity stimuli does not follow directly from decreased sensitivity, but instead reflects an independent pathology.     

The Bjornstad syndrome (sensorineural hearing loss and pili torti) disease gene maps to chromosome 2q34-36

We report that the Bjornstad syndrome gene maps to chromosome 2q34-36. The clinical association of sensorineural hearing loss with pili torti (broken, twisted hairs) was described >30 years ago by Bjornstad; subsequently, several small families have been studied. We evaluated a large kindred with Bjornstad syndrome in which eight members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits. A genomewide search using polymorphic loci demonstrated linkage between the disease gene segregating in this kindred and D2S434 (maximum two-point LOD score = 4.98 at theta = 0). Haplotype analysis of recombination events located the disease gene in a 3-cM region between loci D2S1371 and D2S163. We speculate that intermediate filament and intermediate filament-associated proteins are good candidate genes for causing Bjornstad syndrome.     

Hereditary deafness in Turkey. Initial results

The authors describe a study in progress to identify Turkish families with hereditary hearing loss and isolate possible responsible disease genes. Due to extreme genetic heterogeneity and limited audiological differentiation of hereditary hearing loss, it is necessary to identify large or small families from genetic isolates to locate loci responsible for hearing loss on a chromosome. To accomplish this goal, the medical records of 3800 children were examined at the ENT Clinic of Ege University between 1975 and 1994. All were suspected of having various hearing impairments. Additionally, students from two schools for the hearing impaired in Izmir and Eskisehir, Turkey were examined. To date, 16 families with syndromal deafness and 55 families with non-syndromal hereditary hearing loss involving two or more affected individuals have been identified and categorized according to the mode of inheritance. The majority (66%) of the non-syndromal families showed an autosomal recessive pattern, 29% an autosomal dominant inheritance and 5% an X-linked mode of inheritance. In the study presented there has been a predominance of affected males versus females and the consanguinity rate was 22%.     

Mondini's dysplasia: a rare cause of recurrent meningitis in childhood

Recurrent meningitis in children may be due to immune deficiency or abnormal communication of CSF spaces with mucosal spaces (basal skull fractures, encephalocele, etc.). We report a 6-year-old boy, suffering from recurrent meningitis and sensorineural hearing loss. CT scans of the temporal bones demonstrated a bilateral Mondini dysplasia. In patients with this congenital inner ear anomaly, recurrent meningitis may be due to a CSF-middle ear fistula. We think that it is necessary to investigate all patients with recurrent meningitis and sensorineural hearing loss with CT scans of the temporal bones.     

Replacement by homologous recombination of the minK gene with lacZ reveals restriction of minK expression to the mouse cardiac conduction system

Surgical approaches to the inner ear with hearing preservation have valuable implications for neurotologic surgery. In a previous study in guinea pigs, we demonstrated that click-evoked auditory brain stem responses (ABRs) were preserved after transection and plugging of 1 or more semicircular canals but were lost after entering the vestibule (Smouha EE, et al. Otolaryngol Head Neck Surg 1996; 114:777-784). A limitation of that study was that click-evoked ABR might not represent the function of the entire cochlea. In this study we used tone-burst ABR to determine thresholds across a broad range of frequencies (2 to 24 kHz) before and after surgical entry into the labyrinth at the lateral semicircular canal, ampulla, and vestibule. Serial measurements were made, and a sham surgery group was used as a control. The results obtained with tone-burst ABR generally agreed with those previously obtained with click stimuli. Toneburst ABR thresholds were similar across most frequencies tested. Transection of the lateral semicircular canal resulted in preservation of ABR thresholds. Ampullectomy had a variable effect on ABR thresholds. Vestibulotomy usually resulted in substantial hearing loss. We conclude that hearing can be preserved across a range of frequencies after selective surgery of the inner ear. Surgical entry into the membranous labyrinth near the vestibule is the critical factor contributing to hearing loss after partial labyrinthectomy.     

Aided speech recognition abilities of adults with a severe or severe-to-profound hearing loss

Adults with severe or severe-to-profound hearing losses constitute between 11% and 13.5% of the hearing-impaired population. A detailed investigation of the speech recognition of adults with severe (n = 20) or severe-to-profound (n = 14) hearing loss was conducted at The University of Melbourne. Each participant took part in a series of speech recognition tasks while wearing his or her currently fitted hearing aid(s). The assessments included closed-set tests of consonant recognition and vowel recognition, combined with open-set tests of monosyllabic word recognition and sentence recognition. Sentences were presented in quiet and in noise at +10 dB SNR to replicate an environment more typical of everyday listening conditions. Although the results demonstrated wide variability in performance, some general trends were observed. As expected vowels were generally well perceived compared with consonants. Monosyllabic word recognition scores for both the adults with a severe hearing impairment (M = 67.2%) and the adults with a severe-to-profound hearing impairment (M = 38.6%) could be predicted from the segmental tests, with an allowance for lexical effects. Scores for sentences presented in quiet showed additional linguistic effects and a significant decrease in performance with the addition of background noise (from 82.9% to 74.1% for adults with a severe hearing loss and from 55.8% to 34.2% for adults with a severe-to-profound hearing loss). Comparisons were made between the participants and a group of adults using a multiple-channel cochlear implant. This comparison indicated that some adults with a severe or severe-to-profound hearing loss may benefit from the use of a cochlear implant. The results of this study support the contention that cochlear implant candidacy should not rely solely on audiometric thresholds.     

Cigarette smoking and hearing loss: the epidemiology of hearing loss study

CONTEXT: Clinical studies have suggested that cigarette smoking may be associated with hearing loss, a common condition affecting older adults. OBJECTIVE: To evaluate the association between smoking and hearing loss. DESIGN: Population-based, cross-sectional study. SETTING: Community of Beaver Dam, Wis. PARTICIPANTS: Adults aged 48 to 92 years. Of 4541 eligible subjects, 3753 (83%) participated in the hearing study. MAIN OUTCOME MEASURES: The examination included otoscopy, screening tympanometry, and pure-tone air-conduction and bone-conduction audiometry. Smoking history was ascertained by self-report. Hearing loss was defined as a pure-tone average (0.5, 1, 2, and 4 kHz) greater than 25-dB hearing level in the worse ear. RESULTS: After adjusting for other factors, current smokers were 1.69 times as likely to have a hearing loss as nonsmokers (95% confidence interval, 1.31-2.17). This relationship remained for those without a history of occupational noise exposure and in analyses excluding those with non-age-related hearing loss. There was weak evidence of a dose-response effect. Nonsmoking participants who lived with a smoker were more likely to have a hearing loss than those who were not exposed to a household member who smoked (odds ratio, 1.94; 95% confidence interval, 1.01-3.74). CONCLUSIONS: These data suggest that environmental exposures may play a role in age-related hearing loss. If longitudinal studies confirm these findings, modification of smoking habits may prevent or delay age-related declines in hearing sensitivity.     

Hearing findings in subjects after meningitis

INTRODUCTION: It was proposed to ascertain the occurrence of individuals who present hearing loss, due to contracting meningitis, as well as to characterize the diagnosed loss as far as the type, degree and the audiometric configuration are concerned. MATERIAL AND METHOD: The methodology used comprised a survey of 949 references of patients attended at the Hearing Disturbance Center of the HPRLLP/USP, in order to select those who presented hearing loss after meningitis and, from the analysis of the hearing evaluation to which they were submitted, achieve the proposed goal. RESULTS AND CONCLUSION: The results indicated a 6.2 percent occurrence as regards hearing loss due to meningitis in relation to other causes and the characteristic of this hearing loss was predominantly sensorineural, symmetrical, al linear and to a profound degree.     

Vein graft in stapes surgery

Sealing the opening of the oval window during stapes surgery is essential; it prevents postoperative complications, such as perilymph fistula and sensorineural hearing loss. In this small series of 269 cases with otosclerosis, tympanosclerosis, and congenital ossicular abnormality, vein grafting was used to seal the opening of the footplate. Hearing improvement after surgery was acceptable, and none had total hearing loss or perilymphatic fistula. World literature from the last half of this century on grafting the oval window is reviewed. Absorbable gelatin sponge (Gelfoam) seems to be causing more complications, so its use is highly discouraged. Temporalis fascia, fat, and perivenous loose areolar tissue have been used by different authors at different times in footplate surgery. The opening created in the oval window during stapes surgery must not be left uncovered.     

Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere's disease

The COCH gene is the only gene identified in man that causes autosomal dominantly inherited hearing loss associated with vestibular dysfunction. The condition is rare and only five mutations have been reported worldwide. All affected families showed a similar progressive hearing loss and vestibular dysfunction. Since Meniere's disease-like symptoms have also been described in some families, it was suggested that COCH mutations might be present in some patients diagnosed with Meniere's disease. In this study, using a Japanese population, we performed a COCH mutation analysis in 23 patients from independent families with autosomal dominant hearing impairment, four of whom reported vestibular symptoms, and also in 20 Meniere's patients. While a new point mutation, A119 T, was found in a patient with autosomal dominant hearing loss and vestibular symptoms, no mutations were found in the Meniere's patients. Like all other previously identified COCH mutations, the mutation identified here is a missense mutation located in the FCH domain of the protein. The current mutation is located in close spatial proximity to W117, in which a mutation (W117R) had previously been associated with autosomal dominant hearing loss. Model building suggests that, like the W117R mutation, the A119 T mutation does not affect the structural integrity of the FCH domain, but may interfere with the interaction with a yet unknown binding partner. We conclude that mutations in the COCH gene are responsible for a significant fraction of patients with autosomal dominantly inherited hearing loss accompanied by vestibular symptoms, but not for dominant hearing loss without vestibular dysfunction, or sporadic Meniere's disease.     

The effect of sulphasalazine on neutrophil superoxide generation in rheumatoid arthritis

Sensorineural hearing loss related to autoimmune disease is a well-recognized condition, although the exact pathophysiologic mechanisms remain unclear. One current theory postulates immune complex-induced interference with blood-labyrinth barrier integrity in the stria vascularis. The C3H/lpr autoimmune mouse was chosen to study the permeability of capillaries in the stria vascularis because this mouse model has demonstrated abnormalities of the stria vascularis and shifts in the auditory brain stem response threshold during active disease. C3H/lpr mice with active disease were compared with younger mice without disease, as well as age-matched C3H/HeJ control mice. The mice were injected with the tracer ferritin and examined by transmission electron microscopy to evaluate the integrity of the capillary tight junctions in the stria vascularis. Four of five mice with active disease were noted to have extensive leakage of ferritin into the perivascular tissues. Neither the young, disease-free autoimmune mice nor the nonautoimmune control mice demonstrated vessel leakage. Thickening of the basement membrane was also noted in the diseased animals. The results imply that active disease leads to a breakdown in the blood-endolymph barrier, which could underlie the hearing loss accompanying autoimmune and other immune diseases.     

An implantable hearing aid for inner ear hearing loss. Short-term implantation of microphone and transducer

A microphone constructed for implantation in the posterior wall of the auditory canal and a piezoelectric transducer serving as the main components of an implantable hearing aid were temporally implanted in five patients during middle ear surgery under local anesthesia. The microphone was positioned beneath the skin of the auditory canal such that it completely covered the microphone membrane. The vibratory element of the transducer was coupled to the malleus in four patients with normal ossicular chains and directly to the stapes in one patient with missing incus. The microphone and transducer were electrically connected with an external battery-driven signal amplifier. Speech material and music were presented in the operation room at a sound level of 65 dB SPL under free-field conditions. The patients had to estimate the quality of speech, music, and their own voice as well as the effects of bone-conducting noises. All patients were able to hear with the system. An intraoperative talk without vision contact was possible without any problems, as was understanding of numerals ("Freiburger Zahlentest"). Perception of music was judged as "clear and undistorted with all broadband component." The estimation was also valid for one patient with a sensorineural hearing loss. One patient declared the music to be "a little of unnatural." Bone-conducted sound was estimated as normal in two patients, better than without an implant in one patient with sensorineural hearing loss, and "somewhat metallic" in another patient. Hearing the own voice was considered "normal" in two cases "monotonous" in one case, and "a little bit roaring" in another case. An amplification factor that can be technically realized in an implantable hearing aid was necessary for one of the patients with sensorineural hearing loss to perceive music at a pleasant volume. On the basis of this study, essential requirements for the construction of a fully implantable hearing aid are fulfilled.     

Brainstem auditory evoked response (BER) in paedaudiological diagnosis (author's transl)

Intention of paedaudiology is to detect and to evaluate hearing loss in children. In new born and small infants, in children with behavioral disorders and multiple disabilities, the clinical techniques of audiometry are often not useful. The brainstem audiometry is a reliable alternative and not so cost intensive and time consuming as it is often assumed. Encouraging is this technique in regard of hearing aids: prevention of attaching unnecessary hearing aids, ruling out not appropriate fitted hearing aids and proper habilitation of unilateral hearing loss.     

Sensorineural hearing loss caused by mitochondrial DNA mutations: special reference to the A1555G mutation

Mutations in mitochondrial DNA, which are maternally inherited, have been thought to be one of the causes of sensorineural hearing loss. Two mitochondrial mutational sites (A1555G, A7445G) have been reported to be responsible for non-syndromic hearing impairments. The A1555G mutation causes increased susceptibility to aminoglycoside antibiotic-induced hearing loss as well as non-syndromic sensorineural hearing loss. Our wide screening study showed that there may be a great number of subjects within the Japanese population who have the A1555G mutation. Recent reports suggest that high-risk populations may exist throughout the world. The aminoglycoside-induced hearing loss associated with a mitochondrial mutation is commonly bilateral, symmetric, high frequency involved, and is sometimes associated with progressive sensorineural hearing loss.     

Otoacoustic emissions in full-term newborns at risk for hearing loss

Distortion product otoacoustic emissions (DPOEs) and click-evoked otoacoustic emissions (CEOEs) characteristics of the normal newborn population have been previously reported in the literature. There is little information about DPOE evaluations in the newborn population at risk for hearing loss. The authors now report the DPOE and/or CEOE data from six full-term subjects at risk for hearing loss or with highly suspected hearing loss. These subjects were less than 1 year of age and at risk for hearing loss secondary to a history of hereditary hearing loss, meningitis, hyperbilirubinemia, and ototoxic drug exposure. Audiometric evaluation included auditory brainstem responses (ABR), behavioral observation audiometry, and tympanometry. The CEOEs and DPOEs were found to be decreased or absent in the subjects with suspected hearing loss secondary to cochlear pathology; they were found to be normal in a subject with a suspected central hearing loss. This study's data suggest that otoacoustic emissions when combined with ABR can provide a frequency-specific evaluation of cochlear function and help determine the anatomic site of a pathologic lesion.     

Low prevalence of hearing aid use among older adults with hearing loss: the Epidemiology of Hearing Loss Study

OBJECTIVES: To measure the prevalence of hearing aid use among older adults with hearing loss and to identify factors associated with those currently using hearing aids. DESIGN: Population-based cohort study. SETTING: The south-central Wisconsin community of Beaver Dam. PARTICIPANTS: A total of 1629 adults, aged 48 to 92 years, who have hearing loss and are participating in the Epidemiology of Hearing Loss Study and the Beaver Dam Eye Study. MEASUREMENTS: A hearing-related risk factor and medical history questionnaire, the Hearing Handicap Inventory for the Elderly (screening version), screening tympanometry, pure-tone air- and bone-conduction audiometry, and word recognition tests were administered by trained examiners using standard protocols. RESULTS: The prevalence of current hearing aid use among those with a hearing loss (pure-tone average > 25 decibels hearing level over 500, 1000, 2000, and 4000 Hertz, worse ear) was 14.6%. The prevalence was 55% in a subset of the most severely affected participants. In univariate analyses, current hearing aid use was associated with age, severity of loss, word recognition scores, self-reported hearing loss, self-perceived hearing handicap, and history of noise exposure. Factors associated with current hearing aid use in multivariate logistic regression models were age, severity of loss, education, word recognition scores, Hearing Handicap Inventory for the Elderly (screening version) score, and self-report of a hearing loss. CONCLUSIONS: Few older adults with hearing loss are currently utilizing hearing aids. Improved screening and intervention programs to identify older adults who would benefit from amplification are needed to improve hearing-related quality of life for this large segment of the population.