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.     

Middle ear pressure and dysfunction of the labyrinth: is there a relationship?

Relationships between middle ear pressure and non-infection-related cochleovestibular dysfunction have been suggested by several authors. According to some data, vertiginous attacks can be prevented by the insertion of a ventilation tube in patients suffering from Meniere's syndrome. The aim of our study was to investigate if the incidence of eustachian tube malfunction and pathologic middle ear pressure is frequent, and if routine implantation of ventilation tubes is reasonable in ears with dysfunctions of the labyrinth, including clinical Meniere's syndrome. So, we determined in our pressure chamber all active and passive parameters of eustachian tube function in 40 patients suffering from Meniere's syndrome, sudden sensory hearing impairment (SSHI), or vestibular neuronitis. Our results disclosed no nonrandom incidence of impaired tubal function among our patients compared to healthy control subjects. Pressure equalization was sufficient in most patients suffering from clinical Meniere's syndrome, and only one patient with vestibular neuronitis presented with a patulous tube. Our results show that impairment of vestibular or cochlear function is not regularly accompanied by eustachian tube dysfunction. Furthermore, no patient reported symptoms while pressure variation was performed. We conclude that variation of middle ear pressure does not usually play a role in the genesis of Meniere's syndrome, vestibular neuronitis, or SSHI. Thus, from our data, we cannot recommend routine implantation of tympanic ventilation tubes in patients suffering from Meniere's syndrome, vestibular neuronitis, or sudden hearing loss.     

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.     

Profound bilateral sensorineural hearing loss during gentamicin therapy

A case of profound bilateral sensorineural deafness occurring during gentamicin therapy is presented. Considerable recovery of the hearing in the right ear was observed but the left ear remained "dead". Electrocochleography confirmed the end-organ nature of the damage. Apart from the profound sudden deafness the unusual feature of this case was the total absence of vestibular effects. The ototoxic effects of gentamicin are discussed including the predisposing factors. While the incidence of ototoxicity with gentamicin is low (2-3%) this figure will only remain acceptable if the use of the drug is restricted to those patients who might die or suffer severe morbidity without it. The factors which make side-effects more likely to occur should always be borne in mind and due attention paid to the monitoring of renal function and serum antibiotic levels.     

Ear manifestations in adolescents after closure of lip-jaw-palate- or isolated palatal clefts

We examined two groups of teenagers who had been surgically treated as small children for cleft palate. Most patients were between 13 and 21 years of age. One group had been looked after by the Dept. of Orthodontics at the University of Erlangen-Nürnberg, the other by the Dept. of Orthodontics at the University of Rostock. There were differences in sequence and time of the surgical closure between the two departments. Additionally, 60% of the people treated in Rostock had a velopharyngoplastic, which was rarely the case in Erlangen. In both groups only a few patients had been seen by an ENT-doctor regularly. Only some patients had been previously treated with tubes. There was one patient in each group with a bilateral, most likely genetically determined, sensorineural hearing loss. In Erlangen we examined 66 teenagers (132 ears). Six ears had been previously treated with one or more tympanoplasties. 10 ears needed further treatment due to a seromucotympanon, adhesions, perforations of the ear drum, suspicion of cholesteatoma or insufficient improvement of hearing after previous tympanoplasty. Another 18 ears showed signs of former inflammations. The control group in Rostock included 63 patients (i.e. 126 ears). 14 of the ears examined had undergone one or more tympanoplasties previously. 13 other ears needed further treatment for seromucotympanon, adhesions, perforations of the ear drum, insufficient improvement of hearing after tympanoplasty or cholesteatoma. Residuals due to prior inflammations were found in another 26 ears. Possible reasons for the different occurrence of middle ear problems in both groups are discussed.     

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.     

Active electronic cochlear implants for middle and inner ear hearing loss--a new era in ear surgery. I: Basic principles and recommendations on nomenclature

Hearing aids have fundamental disadvantages: (1) stigmatization of the patient; (2) the sound is often found to be unsatisfactory due to the limited frequency range and undesired distortion; (3) in many patients, the ear canal fitting device generally necessary leads to an occlusion effect; (4) acoustic feedback when amplification is high. Conventional hearing aids transmit sound into the ear canal via a small microphone. Sound has the disadvantage of requiring high output sound pressure levels for its transmission. This along with the necessary miniaturization of the loudspeaker as well as the resonances and reflections in the closed ear canal contribute to the disadvantages mentioned. In contrast, implantable hearing aids do not make sound signals but micromechanical vibrations. An implantable hearing aid has an electromechanical transducer instead of the loudspeaker of a conventional hearing aid. The hearing signal does not leave the transducer as sound but as a mechanical vibration which is directly coupled to the auditory system bypassing the air. This implantable hearing aid is either coupled to the tympanic membrane, the ossicular chain, the perilymph of the inner ear, or the skull. An implantable hearing aid is expected to have: 1 Better sound fidelity than a hearing aid 2 No ear canal fitting device, free ear canal 3 No feedback 4 Invisibility Requirements on electronic hearing implants designed for patients with conductive hearing loss differ from those on implants for sensorineural hearing loss. Conductive hearing loss requires the implant to replace the impedance transformation, thus being an impedance transformation implant (ITI). In various respects, the demands on an ITI are lower than the demands on an electronic hearing aid for patients with sensorineural hearing loss. The latter are mostly patients with a failure of the cochlea amplifier (CA). A damage to the CA is clinically discernible by a positive recruitment and loss of otoacoustic emissions (OAE). Since these patients form the majority of cases with sensorineural hearing loss, an active hearing implant for such patients should partially replace the function of the CA. Therefore, the suggestion is to refer to a CAI (cochlea amplifier implant). The implant expressions ITI (for patients with conductive hearing loss) and CAI (for patients with sensorineural hearing loss) used in this context allow nomenclatural association with the CI (cochlear implant) for complete inner ear failure as well as with the BSI (brainstem implant) in the case of hearing nerve failure.     

Sudden deafness and Barlow disease

From January 1993 to December 1994 twelve patients were evaluated for sudden hearing loss. The median age was 49 years with a range of 18 to 71. All had severe or profound initial hearing loss. The incidence of bilateral disease was 25%. Total deafness occurred in five (33%) ears. Nine (75%) patients had vestibular symptoms and eight (67%) admitted experiencing tinnitus. Two-dimensional echocardiography revealed mitral prolapse in eight (67%) patients; another patient showed moderate to severe ischemic left ventricular dysfunction with apical aneurysm.     

Total unilateral deafness in children. Etiologies and long-term consequences

A retrospective study of 72 children with a profound (average hearing loss > 90 dB) unilateral sensorineural hearing loss (PUSNHL) was carried out in the ENT department at the Rouen hospital in 1988 and 1994. This study intended to investigate the etiologies, how it was recognized, the age of the onset and the results of the medical evaluation of these PUSNHL. We found, in 2 cases, that vestibular lesions of the healthy ear were present before the onset of a hearing loss on this side, turning the PUSNHL into a bilateral deafness. We tried to correlate the etiologies, age of onset of the PUSNHL, status of the opposite ear and possible scholar problems in the group of the 42 children followed-up for more than 2 years (mean follow-up time = 7 years). Among children suffering from PUSNHL, we specified a group with higher risk of school failures, allowing ENT physicians to propose more accurate management to these children.     

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.     

Long-term hearing results following vestibular surgery in Meniere's disease

OBJECTIVES: The purpose of this study is to evaluate the long-term hearing changes following vestibular surgery in patients with Meniere's disease. Study DESIGN: This is a retrospective analysis of patients operated on in a tertiary referral center setting. METHODS: Preoperative, postoperative, and 3- to 9-year postoperative audiograms were analyzed in two patient groups. Twenty-one patients underwent posterior fossa vestibular neurectomy (VN) and five, mastoid endolymphatic sac decompression and shunt (ELS). All frequencies, four-frequency pure-tone averages (PTAs), spondee thresholds, and speech recognition scores were compared for operated ear against nonoperated ear of VN subjects. The results were subjected to a covariance analysis. VN and ELS patients whose hearing deteriorated from "serviceable" (PTA < or =70 dB hearing level) and speech recognition > or =30%) to nonserviceable status were compared using nonparametric statistics. RESULTS: Progressive hearing loss beyond the rate of change of the normal contralateral ear was evident in all patients. Serviceable hearing dropped from 81% to 43% of patients an average of 4 years following VN. CONCLUSIONS: VN patients have significant hearing deterioration over time in the operated ear. This finding suggests that continued postoperative medical management is necessary for patients undergoing VN.     

Immune-mediated inner ear disease: report of clinical cases

Many clinical and experimental studies have obtained evidence of immune-mediated inner ear disease. Discrepancies between theories of the mechanisms of injury to the inner ear and the laboratory tests that identify it mean that the diagnosis is based on clinical symptoms and a positive response to treatment. We report four cases of immune-mediated inner ear disease characterized by endolymphatic hydrops, fluctuating hearing loss, sudden deafness (first symptom of primary Sj?gren's syndrome), and rapidly progressive sensorineural hearing loss.     

Hearing and Sj?gren's syndrome

Immune sensorineural hearing loss is manifested in several systemic immune diseases. Although hearing loss has been previously documented in patients with Sj?gren's syndrome (SS), the effect of SS on hearing is unclear. This prospective study was designed to assess the presence of hearing loss in 14 patients with SS and, if sensorineural hearing loss was present, to determine if the hearing loss was immune-mediated. Patients were evaluated with basic audiologic tests as well as for cellular immune inner ear reactivity as measured by the lymphocyte transformation test (LTT). Three patients had evidence of sensorineural hearing loss. Two patients had a positive LTT without evidence of sensorineural hearing loss. This preliminary study suggests that SS may not directly cause sensorineural hearing loss, immuno-mediated or otherwise.     

Hypercholesteremia and hyperfibrinogenemia in sudden deafness

BACKGROUND: The pathogenesis of sudden hearing loss has not been elucidated as yet. Insufficient perfusion of the cochlea due to an increased blood viscosity, microthrombosis, or altered vasomotion are assumed. Hypercholesterolemia and hyperfibrinogenemia are frequently observed in patients with sudden sensorineural hearing loss. The aim of this study was to investigate the incidence of hypercholesterolemia and hyperfibrinogenemia in patients suffering from sudden hearing loss compared to normal controls. In an intervention study the impact of drastic lowering of plasma cholesterol and fibrinogen by a selective extracorporal apheresis was studied. METHODS: In a case-control study of 23 patients suffering from sudden hearing loss, plasma cholesterol and fibrinogen levels as well as erythrocyte aggregation and plasma viscosity were determined. Seven sudden hearing loss patients from this group were treated with H.E.L.P. apheresis, an extracorporal procedure removing fibrinogen and idl-cholesterol from plasma. RESULTS: Plasma fibrinogen and cholesterol levels were higher in sudden hearing loss patients, leading to significantly elevated values of erythrocyte aggregation and plasmaviscosity. Six out of the seven patients treated with a single H.E.L.P. apheresis immediately showed an improvement of auditory thresholds. CONCLUSIONS: We conclude that hyperfibrinogenemia and hypercholesterolemia may contribute to the clinical event of sudden hearing loss. Our study shows for the first time that acute and drastic removal of plasma fibrinogen and low density lipoproteins can be an effective clinical tool in the treatment of patients with sudden hearing loss.     

Acute autoimmune sensorineural hearing loss associated with Crohn's disease

We report the sudden onset of bilateral hearing loss in a patient with Crohn's disease while the intestinal disease was quiescent. Antibodies directed against collagen type II were detected in the serum. Dramatic improvement of his hearing was observed under corticosteroid therapy. Diagnosis of autoimmune sensorineural hearing loss was established. This condition could be considered as an extraintestinal manifestation of Crohn's disease. Early diagnosis is mandated, as prompt and intensive treatment with corticosteroids and/or immunosuppressive drugs is effective and could prevent irreversible hearing loss.     

Disorders of adult personality and behaviour (F6). Results from the ICD-10 field trial of the Diagnostic Criteria for Research in German-speaking countries

Immune-mediated inner ear disease, first described by McCabe in 1979, typically presents with an idiopathic, rapidly progressive bilateral sensorineural hearing loss. The course of the hearing loss occurs over weeks to months. It may occur in both sexes and at a variety of ages, but is most common in middle-aged females. It may be accompanied by tinnitus, Meniere's-like vertigo, or more commonly, ataxia or unsteadiness. Approximately 30% of patients will have associated systemic immune-mediated disease. Although refinements in laboratory tests for specific inner ear antigens are being made, and non-specific laboratory indicators of inflammatory or systemic immune disease may be useful in confirming the diagnosis, the most important diagnostic finding is the improvement in hearing seen with a trial of immunosuppressants. This report includes a typical evaluation of the patient with suspected immune-mediated inner ear disease and an illustrative case. Sensorineural hearing loss due to immune-mediated inner ear disease, although unusual as a cause of hearing loss, is important to recognize because early diagnosis and treatment can have a marked effect on the clinical outcome.     

Predicting acute denervation in carpal tunnel syndrome

OBJECTIVE: Defects of the cochlear modiolus have been found to be associated with most cases of large vestibular aqueduct. The clinical significance of these modiolar defects has not been studied previously. The purpose of this article is to correlate clinical (functional) parameters, such as hearing outcomes, with the severity of the radiographic findings in these dysplastic inner ears. STUDY DESIGN: The study design was a retrospective chart review, supplemented with telephone interviews and clinic visits. SETTING: The study was conducted at an academic, tertiary care center. PATIENTS: Thirty consecutive patients with large vestibular aqueducts participated. RESULTS: Scores of modiolar deficiencies yielded inconsistent correlations with hearing loss. Vestibular aqueduct morphology and thickness correlated very strongly with the severity of hearing loss. CONCLUSIONS: These observations support the hypothesis that large vestibular aqueduct-related hearing loss may be caused by transmission of subarachnoid pressure forces into the inner ear. However, the thickness and morphology of the vestibular aqueduct may simply be markers for more subtle cochlear dysplasia manifest by modiolar deficiency.     

On the role of the olivocochlear bundle in hearing: 16 case studies

Earlier we presented data (Scharf et al. (1994) Hear. Res. 75, 11-26) from a young patient (S.B.) who had undergone a vestibular neurotomy, during which the olivocochlear bundle (OCB) was severed. Those data are complemented by measurements on 15 other patients-some like S.B. with normal audiometric thresholds, none with a loss greater than 35 dB at experimental frequencies. Comparisons of performance for the same ear before and after surgery or between the operated and healthy ears do not provide evidence that the lack of OCB input impairs the following psychoacoustical functions: (1) detection of tonal signals, (2) intensity discrimination, (3) frequency selectivity, (4) loudness adaptation, (5) frequency discrimination within a tonal series, (6) in-head lateralization. Data on single-tone frequency discrimination are equivocal. These mostly negative results apply to listening both in the quiet and, where relevant, in noise. The only clear change in hearing after a vestibular neurotomy is that most patients detect signals at unexpected frequencies better than before. This change suggests an impaired ability to focus attention in the frequency domain. Although limited in scope, our finding that human hearing without OCB input is essentially normal agrees with much of the relevant literature on animal behavior and with the patients' self-reports.     

Intensity and frequency discrimination monitored by evoked response audiometry: clinical application

Delta 1 and delta f repetitive stimuli superimposed on continuous 0.5-, 1- and 4-kHz carrier tones were administered to 20 normally hearing persons, 8 adults with sensorineural hearing loss, 10 adults with conductive hearing loss and 22 children subjected to evoked response audiometry (ERA) for assessment of hearing acuity. The intensity modulation depth ranged from + 1 to 10 dB. In the subjects with normal hearing and in the adult patients, three carrier tone levels were used whenever possible: 20, 40, and 60 dB SL. The frequency modulation depth ranged from -1 to -10% of the carrier tone frequency. The same levels for the carrier tone were used: 20, 40, and 60 dB SL. With a carrier tone level of 20 dB SL, a clear-cut vertex response was noted in 80% of the tests carried out in the normal adults with 5-dB delta i stimuli and with delta f stimuli of -3 to -5% in magnitude; the same figures are valid for the patients with sensorineural and conductive hearing loss. The patients with sensorineural hearing loss showed a tendency to give vertex responses with lower delta i stimuli than subjects with normal hearing. However, this difference was not statistically significant. According to our results, the children tested can be roughly divided into two groups, the first including subjects with responses to delta i stimuli of +5 and +10 dB and delta f stimuli of -5% and -10%; the second group including subjects with no clear-cut responses to these stimuli.     

Surgical management of the congenital atresia of the ear

This study presents a 15 year experience of surgical treatment in 47 patients with congenital atresia of the ear. Emphasizing on the importance of pre-surgical evaluation of the patients for a better prognostic indicator. The ages of 40 patients were between 5-15 years. 24 patients (61%) had complete atresia and 23 (49%) incomplete. 27 patients (57%) with bilateral disease and 20 (43%) unilateral. Auditory test before surgery showed a conductive hearing loss of approximately 60 decibels (dB) and one case with total sensorineural hearing loss. There was an auditory average gain of 28.3 dB. 3 patients (6.4%) had associated cholesteatoma. Complications presented: Closure of the external auditory canal in 6 patients (12.7%), and facial nerve paralysis in 2 patients with recovery. The different surgical technics are described. In conclusion, for this type of surgery, combination between the otologist and plastic surgeon should be done for better results.