Early identification and intervention for children who are hearing impaired

Early identification of and intervention for all children who have hearing impairments remain unattained goals in the United States. Physicians typically are the first persons to obtain the medical and family history of infants and children and are the primary professionals confronted with parental concerns about hearing loss. Heightened awareness of the common causes of hearing loss in infants and children can facilitate prompt and appropriate referrals to audiologists when hearing loss is suspected. A strong and interactive relationship between physician and audiologist is needed to attain the common goals of providing the earliest and best possible diagnosis of and optimal management for hearing impaired pediatric patients.     

Hearing loss. The physician's responsibility

Hearing loss is a very important and common problem in the United States, affecting 13 to 14 million people. It is primarily a medical problem, requiring diagnosis and, when indicated, treatment by a physician, preferably in collaboration with an audiologist. History taking is the most important part of the diagnostic process, followed by a physical examination with special attention to the head, neck, and ears. The primary object of the workup is to exclude all possible extrinsic causes of hearing loss. Intrinsic or genetic deafness is a diagnosis of exclusion. After the hearing loss has been classified as congenital or delayed and as genetic or nongenetic, a differential or working diagnosis can be made in all cases and a specific diagnosis in most cases. The hearing loss must then be identified as stable, progressive, or fluctuant over time. Follow-up at least every two years is important to check for possible changes in status.     

Physical diagnostic procedures: whispered speech and tuning fork test]

Hearing impairment occurs in 10% of the Dutch population and may lead to major communication problems and even social isolation. A good method to detect hearing loss in a general practice is the screening audiometer. The treating physician may further use the whispered speech test and tuning fork tests which together give a good impression of the severity and the nature of the hearing loss if any. The whispered speech test is best performed in the standardized form according to the guideline 'Slechthorendheid' [hardness of hearing] of the Dutch College of General Practitioners (NHG), in which certain combinations of letters are recommended. The tuning fork tests according to Rinne and Weber indicate a difference in perception and conduction deafness, and are decisive for any hearing asymmetry. Use of the whispered speech test and of the tuning fork tests is recommended for adults and children from the age of 7.     

Fluctuant perception hearing loss after hepatitis B vaccine

A 42 year old physician doctor presented with temporary tinnitus after a first dose of a hepatitis B vaccination (Engerix B*). One month after, a fluctuant sensori-neural hearing loss with tinnitus appeared few hours after a second dose of hepatitis B vaccination (Engerix B*). The auditory thresholds varied from 45 dBHL and 0 dBHL for low frequencies below 1.5 kHz. The fluctuant hearing loss and tinnitus disappeared after a 6 month course.     

Aging, hearing acuity, and the attentional costs of effortful listening.

A dual-task interference paradigm was used to investigate the effect of perceptual effort on recall of spoken word lists by young and older adults with good hearing and with mild-to-moderate hearing loss. In addition to poorer recall accuracy, listeners with hearing loss, especially older adults, showed larger secondary task costs while recalling the word lists even though the stimuli were presented at a sound intensity that allowed correct word identification. Findings support the hypothesis that extra effort at the sensory–perceptual level attendant to hearing loss has negative consequences to downstream recall, an effect that may be further magnified with increased age. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Hearing loss secondary to lumbar puncture

Hearing complaints have been described in patients after lumbar puncture, although other symptoms like headache, nausea, and dizziness can easily obscure hearing disorders. Reduced cerebrospinal fluid pressure after lumbar puncture may explain the origin of this vestibulocochlear dysfunction. Two cases of hearing loss and tinnitus that appeared hours after otherwise uncomplicated diagnostic spinal puncture are reported. The pure tone audiogram showed a 40-50 dB bilateral sensorial hearing loss in the lower frequencies. Hearing recovered and tinnitus disappeared entirely within 7-10 days. Since permanent hearing loss has been reported after diagnostic or therapeutic lumbar puncture, we should be aware of the possibility of this adverse effect.     

Oncocytic hepatocellular carcinoma with numerous globular hyaline bodies

The current approach to the management of physician resources in Canada needs to be re-examined by all concerned. Canada is about to enter a phase of accelerating depletion of physicians as the result of two separate and evolving circumstances. Because of the unusually large number of physicians who graduated from Canadian medical schools in the late 1960s and early 1970s, a significantly larger than usual number of practising physicians will reach their normal retirement age in the decade ahead. In addition, if the recent surge in the emigration of Canadian physicians continues, the loss of so many physicians will exaggerate the impact of the expected increase in retirements. Therefore, the decision to cut medical school class sizes in the 1990s would have been more suitable in the early 1980s. Existing physician work force policies may be leading to unexpected or undeclared consequences for health care across Canada. On the basis of current trends, the author concludes that policy makers now should reconsider current physician workforce policies in anticipation of a possible shortfall of physicians beginning in the early decades of the next century.     

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.     

Patient-physician sexual involvement: a Canadian survey of obstetrician-gynecologists

OBJECTIVE: To determine obstetrician-gynecologists' (ob-gyns') awareness of and experience with sexual abuse of patients and former patients and their opinions about appropriate consequences. DESIGN: Mailed survey. SETTING: Canada. PARTICIPANTS: All 792 members of the Society of Obstetricians and Gynaecologists of Canada (SOGC); 618 (78%) responded. Approximately half of all ob-gyns in Canada belong to the SOGC. MAIN OUTCOME MEASURES: Knowledge of sexual involvement by an ob-gyn colleague with a patient or former patient (as defined by the respondents and by the College of Physicians and Surgeons of Ontario [CPSO]), self-report of such involvement, attitudes toward physician sexual abuse, desirable length of time a physician should wait before seeing a former patient in a situation that could lead to a sexual encounter, suggested consequences of sexual abuse. RESULTS: Overall, 10% of the respondents indicated that they knew about another ob-gyn who at some time had been sexually involved with a patient. In all, 3% of the male respondents and 1% of the female respondents reported sexual involvement with a patient; the corresponding proportions of those who reported having been accused of sexual abuse by a patient were 4% and 2%. Significantly more of the female ob-gyns than of their male counterparts (37% v. 19%) reported awareness of a colleague's sexual involvement with a patient that would meet the CPSO's definition of sexual impropriety, transgression or violation. Most of the respondents felt that the consequence of proven sexual impropriety should be reprimand and fine (chosen by 33%) or rehabilitation without loss of licence (28%). Most of the physicians supported loss of licence for proven sexual transgression (57%) or proven sexual violation (74%), but fewer felt that loss of licence should be permanent for these types of abuse (4% and 24% respectively). The female ob-gyns supported stronger sanctions against sexual transgression and sexual violation than the male ob-gyns. A wide range of opinion was seen regarding the propriety of sexual relationships with former patients. CONCLUSIONS: Ob-gyns have varied opinions about how sexual abuse of patients should be defined and how it should be sanctioned. There is a discrepancy between proposed public policy and the beliefs of physicians to whom the policy is to be applied.     

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.     

Hearing loss after erythromycin therapy

We describe the case of a patient who developed bilateral loss of hearing during intravenous treatment with erythromycin. Predisposing factors may have been reduced renal and hepatic function and treatment with diuretics and aminoglycosides. Recovery was late, but hearing was probably restored to almost pre-treatment level. Patients receiving high doses of erythromycin should, as a routine, be questioned about hearing loss and tinnitus.     

Management of otitis media in a high risk population

BACKGROUND: Rural and remote Aboriginal and Torres Strait Islander children have extremely high rates of otitis media and hearing loss. Despite considerable evidence, clinical practice continues to vary. This may be partly related to the failure of recent guidelines to be explicit about which factors should influence decision making. OBJECTIVE: To provide rural and remote GPs caring for young Aboriginal and Torres Strait Islander children with an evidence based guide to the principles that determine the clinical management of otitis media and hearing loss. DISCUSSION: While population health strategies are extremely important, the GP also needs to be able to advise families of affected children of the benefits and risks of the medical, surgical and audiological interventions available. The key to this process is the ability to distinguish between suppurative and non-suppurative disease, and a familiarity with the natural history and the likely hearing loss associated with different disease states in high risk populations.     

Symptomatic treatment of neurolathyrism with tolperisone HCL (Mydocalm): a randomized double blind and placebo controlled drug trial

Birth hypoxia, asphyxia and ischemia have often been thought to be major causes of early hearing loss or deafness. The purpose of the present review is to focus on the role of these particular factors for perinatal auditory disorders. On the whole, only a small proportion of neonatal hearing loss is caused by perinatal factors. The exact etiology of neonatal hearing loss in children with complicated deliveries is difficult to evaluate due to the large number of causative factors that might be involved. After reviewing the literature covering the past 15-20 years, it is not possible to say that we understand the relative importance of different factors and their interactions. However, in the majority of studies, birth asphyxia is not correlated with hearing loss in babies with complicated deliveries Prolonged artificial ventilation, the presence of severe hypoxic ischemic encephalopathy or persistent pulmonary hypertension are important factors. The brain is more susceptible to anoxia than the ear and both are more likely to be damaged after prolonged pre-, peri- and postnatal hypoxia-ischemia than pure hypoxia during delivery. Perinatal hypoxia is more likely to cause a temporary hearing loss than a permanent one. Preterm babies are more vulnerable than term babies. The total number of risk factors, e.g. medicated by total length of stay in the neonatal intensive care unit and length of artificial ventilation, is the best predictor of risk for hearing loss of perinatal origin. The similarities between hearing loss and cerebral palsy are pointed out; only 8% of the cases of cerebral palsy are considered to be caused by conditions during delivery.     

Meniere's disease of syphilitic etiology

Congenital or acquired syphilis is the cause of Meniere's disease in six percent of all cases. The pathogenesis is endolymphatic hydrops and osteitis of the otic capsule which produces the characteristic fluctuating hearing loss, tinnitus and ear pressure associated with episodic spells of vertigo. The ear symptoms are, at first, typical of Meniere's disease of other etiologies. Failure to establish the specific etiologic diagnosis can result in unnecessary surgical treatment or inappropriate medical therapy. In some cases, if a steroid, the specific treatment, is not given promptly, hearing can be quickly and permanently lost. The etiology is confirmed by obtaining a reactive fluorescent treponema antibody absorption (FTA-abs) test. The clinical features and natural history of Meniere's disease of syphilitic etiology are unique. Ear symptoms often begin in the fifth decade of life, first in one ear and, after a few years, involving the second ear. Reduced or absent caloric responses are characteristic. When hearing loss is severe and sudden, this condition represents a true medical emergency and should be treated with prompt administration of steroids. An endolymphatic subarachnoid shunt operation is occasionally necessary as adjunctive management. An analysis of 34 cases shows that long-term use of steroids can maintain effective hearing for more than 20 years.     

Unilateral hearing loss due to a rhabdomyoma in a six-year-old child

A case report of a six-year-old child is presented, who had had a unilateral sensorineural hearing loss for several years. Because of impairment in the ABR as well as in the caloric testing a MRI and CT scan were performed. A 17 mm tumour in the cerebellopontine angle (CPA) was detected, which after suboccipital surgery proved to be a rhabdomyoma. This tumour has not been described before in the CPA. Unilateral sensorineural hearing loss should, at all ages, be an indication for further (radiodiagnostic) investigations.     

The rehabilitation of conductive hearing impairment

Conductive hearing loss (CHL) usually is amenable to surgical correction. At times, patients with CHL may not be candidates for surgical correction, or they may refuse to undergo the procedure. In such a situation, the patient should be encouraged to try hearing aids, either conventional or bone conduction, depending on the pathologic condition. After a discussion of bone-conduction implantable hearing aids, the article discusses congenital aural atresia, otosclerosis, and the management of CHL after infratemporal fossa approach and transtemporal approaches.     

Growth behavior of the 2 f1-f2 distortion product otoacoustic emission in tinnitus

High-resolution hearing threshold and 2 f1-f2 distortion product otoacoustic emission (DP) were measured with the same in-the-ear sound probe and same calibration at 51 frequencies between 500 and 8000 Hz in 39 sensorineural hearing loss ears associated with tinnitus. Using a primary tone setting L1 = 0.4L2 + 39 that accounts for the nonlinear interaction of the two primary tones at the DP generation site at f2, DPs were elicited in a wide range from L2 = 65 to 20 dB SPL. We failed to find a uniform DP behavior in the 39 tinnitus ears tested. Seventeen of them behaved like impaired ears without tinnitus. In these ears a linearized DP growth was observed where the DP level decreased and the slope of the DP I/O functions steepened with increasing hearing loss and as a result both the DP level and the DP slope strongly correlated with hearing threshold. The other population, 22 tinnitus ears, exhibited a poor or even inverse relationship between DP level and hearing threshold, i.e., displayed an increase of DP level with increasing hearing loss. Despite the severe hearing loss but due to the high level, DPs could be recorded well in the frequency range that corresponded to the appearance of the tinnitus. The DP slope, however, increased with increasing hearing loss and, therefore, did still correlate with hearing threshold revealing pathological alteration. The data suggest that the DP level alone is hardly capable of assessing hearing impairment in tinnitus ears and may even be misleading. Thus just the DP slope seems to be the only reliable indicator of cochlear malfunction around the tinnitus frequency. The observed nonuniform DP behavior suggests different cochlear impairments in tinnitus ears. In those ears where the DP level decreases and the slope of the I/O functions increases with hearing loss, cochlear sensitivity and tuning are supposed to be diminished. In those ears where the DP level increases with increasing hearing loss, a reinforced mechanical distortion is hypothetized to be generated by cochlear hyperactivity that can be the source of both the abnormally high DP level and the tinnitus.     

Surgery for intractable vertigo: Chiang Mai experience

If medical therapy fails (6-12 months or more), careful consideration of surgical therapy should be followed with; the patient's hearing, severity of symptoms, age, and occupation. Cochleosacculotomy is reserved for elderly Meniere's patients with poor health, poor hearing and good vestibular function. Endolymphatic sac shunt should be considered as the first procedure for disabled Meniere's patients with aidable hearing and may also be used in those patients with bilateral Meniere's disease. Retrolabyrinthine vestibular nerve section (RLVNS) is indicated for patients with disabling vertigo and normal or aidable hearing. It could be done for all peripheral vertigo and for failed endolymphatic sac procedure. A destructive procedure, such as labyrinthectomy, should be a procedure of choice in a patient who presents with peripheral incapacitating vertigo and nonserviceable hearing loss in the solely affected ear. Again, patients with failed cochleosacculotomy, endolymphatic shunt then go on to receive either labyrinthectomy or RLVNS, based on residual hearing. To obtain a satisfactory surgical result, the surgeon must be precise in selecting the patient with the right disease at the right time.