Hearing and otopathology in Crouzon syndrome.

OBJECTIVES: To better establish the incidence and types of otologic and auditory abnormalities in patients with Crouzon syndrome. STUDY DESIGN: Retrospective chart review of the otologic and auditory findings of patients diagnosed with Crouzon syndrome who were seen at our institution between 1978 and 1994. METHODS: Charts were reviewed and data recorded on patient sex, family history, appearance, auricular abnormalities, auditory findings, history of otologic disease, and follow-up. RESULTS: Nineteen patients were identified with the diagnosis of Crouzon syndrome: 12 males and 7 females. Twelve cases represented spontaneous mutations. Eight patients had abnormalities involving the external ear: from malalignment of the pinna (6 patients) to external auditory canal atresia (1 patient). Ten patients had documented hearing loss: 4 with conductive hearing loss, 2 with a mixed hearing loss, and 4 with a sensorineural hearing loss, the etiologies of which ranged from ossicular fixation and serous otitis media to unknown sensorineural deficits. CONCLUSIONS: Patients with Crouzon syndrome can exhibit various pathological features of the ear. Although external malformations are unusual, middle ear disease and hearing loss are common. We advocate close otologic and audiologic follow-up in these patients and note a higher frequency of sensorineural hearing loss than previously reported. Recent genetic advances may allow more accurate and earlier diagnosis of this syndrome.     

Auditory function in patients with surgically treated superior semicircular canal dehiscence.

OBJECTIVE: To characterize preoperative and postoperative audiologic findings in patients with superior semicircular canal dehiscence syndrome. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Patients with documented superior semicircular canal dehiscence syndrome (according to history, vestibular testing, and high-resolution computed tomography imaging) who underwent surgical repair of their dehiscence. INTERVENTION: Middle fossa craniotomy for superior semicircular canal plugging and/or resurfacing. MAIN OUTCOME MEASURES: Audiologic testing both before and after surgery with pure-tone threshold measurements of air and bone conduction. RESULTS: Twenty-nine subjects underwent surgical repair of superior semicircular canal dehiscence. Overall, there were no statistically significant differences by paired t test in hearing before or after surgery, in either air-conduction or bone-conduction thresholds, for 19 patients that had no previous surgical history. At least partial closure of air-bone gap was achieved in five patients. One patient with previous stapes surgery had significantly worse hearing both before and after canal repair compared with those without previous surgery. Two patients who had undergone previous middle fossa surgery with incomplete resolution of symptoms developed sensorineural hearing loss after revision surgery. Previous middle-ear exploration and tympanostomy tube placement did not seem to affect audiologic outcomes. Surgical hearing results did not differ according to method of canal repair (plugging versus resurfacing). CONCLUSION: Primary middle fossa repair of superior semicircular canal dehiscence is not associated with sensorineural hearing loss and, in some cases, can lead to normalization of conductive hearing loss. Revision middle fossa repair or previous stapes surgery may be associated with postoperative sensorineural hearing loss.     

Otologic disease in turner syndrome.

OBJECTIVE: This study involved the assessment of the otologic and audiologic characteristics of a group of patients with Turner syndrome. STUDY DESIGN: Prospective study consisting of a questionnaire (77 of 123 responders) and an otologic and audiologic evaluation in patients with Turner syndrome (41 participants). SETTING: Tertiary academic medical setting. PATIENTS: Children, adolescents, and adults with Turner syndrome (median age, 24 yr). INTERVENTIONS: Otomicroscopy, audiometry, immittance measurements, and diagnostic imaging. RESULTS: Otologic disease is an important characteristic in Turner syndrome. Sixty-six percent of the patients studied via the questionnaire reported a history of chronic or recurrent middle ear disease. Analysis of audiometric data in 40 patients tested reveals an equal amount of normal ears (38.8%) and pure sensorineural ears (38.8%), each constituting approximately one-third of the patient population. Pure conductive losses represent only one-fifth (21.3%) of auditory abnormality encountered. CONCLUSIONS: Careful follow-up during early childhood of children with Turner syndrome is necessary to detect middle ear disease and prevent sequelae. However, long-term periodic review is mandatory even after resolution of middle ear disease to detect sensorineural hearing loss.     

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder

ObjectiveThe focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors.Study designRetrospective AudGen Database analysisSettingTertiary academic referral centerPatientsPediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS).InterventionsAppropriate audiologic, otologic, and demographic data were recorded.Main outcome measureSeventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented.Results57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss.ConclusionThis study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.     

Noncongenital hereditary hearing loss in children. Prospective documentation

Younger siblings of children with sensorineural hearing loss of possible hereditary cause underwent interval audiologic examination. Seven siblings (in unrelated families) were found to have progressive sensorineural hearing loss despite early audiograms documenting normal hearing levels for age. Continued testing of these children allowed for early identification and intervention. We advocate regular otolaryngologic and audiologic follow-up even after normal audiologic assessments are made for younger siblings of children with documented sensorineural hearing loss, unless a definite nongenetic origin of the hearing loss in the older child is known. Recessive sensorineural hearing loss with onset in infancy or childhood may present with no antecedent family history and with normal behavioral audiograms early in life.     

Audiologic and vestibular findings in a sample of human immunodeficiency virus type-1-infected Mexican children under highly active antiretroviral therapy

OBJECTIVE: There is little information about audiologic and vestibular disorders in pediatric patients infected with the Human Immunodeficiency Virus type-1 (HIV-1). The aim of this study was to evaluate audiologic and vestibular disorders in a sample of HIV-1-infected children receiving Highly Active Antiretroviral Therapy. METHODS: Patients underwent pure tone audiometry, speech discrimination testing, auditory brainstem responses, electronystagmography, and rotatory testing. HIV-1 viral load and absolute CD4+ cell counts were registered. RESULTS: Twenty-three patients were included, aged 4.5 years (median, range 5 months to 16 years). Pure tone audiometry was carried out in 12 children over 4 years of age: 4 (33%) showed hearing loss, 2 were conductive. Auditory brainstem responses were measured in all 23 patients, suggesting conductive hearing loss in 6 and sensorineural hearing loss in 2. Most patients with conductive hearing loss had the antecedent of acute or chronic suppurative otitis media but with dry ears at the time of evaluation (p=0.003). Abnormal prolongations of interwave intervals in auditory brainstem responses were observed in 3 children (13%, 4 ears), an abnormal morphology in different components of auditory brainstem responses in 4 (17.4%, 7 ears), and abnormal amplitude patterns in 11 patients (48%, 17 ears). Vestibular tests were abnormal in all six patients tested, with asymmetries in caloric and rotatory tests. Although differences were not significant, in general, audiologic abnormalities were more frequent in patients with more prolonged HIV-1 infections, higher viral loads, or lower absolute CD4+ cell counts. CONCLUSIONS: Conductive hearing loss associated with previous otitis media events, abnormalities in auditory brainstem responses suggesting disorders at different levels of the auditory pathways, and unilateral vestibular hyporeflexia were frequent findings in our sample of HIV-1-infected children under Highly Active Antiretroviral Therapy. These findings suggest that HIV-1-infected children should be submitted to audiologic and vestibular evaluation as early as possible in order to reduce their impact on the psychosocial development of these patients.     

An investigation of the audiologic handicap associated with unilateral sudden sensorineural hearing loss

OBJECTIVE: To determine the incidence of tinnitus and associated handicap after unilateral sudden sensorineural hearing loss (SSNHL); in addition, to determine the hearing handicap experienced as a consequence of such a loss. STUDY DESIGN: Identification of patients and determination of demographic and audiologic data by retrospective case review; determination of handicap and distress by postal questionnaire. SETTING: Teaching hospital department of otolaryngology. PATIENTS: Thirty-eight patients were identified as having been treated for a unilateral sudden sensorineural hearing loss in the period 1988 through 1997. Of those, 21 (55.3%) replied to the questionnaire. MAIN OUTCOME MEASURES: Audiometric data at admission and at 4-week follow-up, Tinnitus Handicap Inventory (THI), visual analogue scales of tinnitus loudness and distress, Hearing Handicap Inventory in Adults (HHIA). RESULTS: The questionnaire responder group did not significantly differ from the questionnaire nonresponder group on demographic nor audiometric variables, and hence were considered to be a representative sample. Tinnitus was present in 14 patients (67%). Hearing handicap was found in 86% of patients (of the 21 questionnaire responders) and tinnitus handicap in 57% (of the 14 with tinnitus). Correlations were found between tinnitus loudness, distress, and handicap. There was no correlation between time elapsed since SSNHL and tinnitus or hearing handicap, nor was there a correlation between the extent of audiometric loss and hearing or tinnitus handicap. A strong negative correlation was, however, found between recovery in audiometry in the first 4 weeks after onset and tinnitus and hearing handicap. The audiometric status of the contralateral ear correlated with hearing handicap. CONCLUSIONS: A majority of patients after unilateral SSNHL have a perceived handicap associated with tinnitus and hearing. Although this condition is an otologic emergency, careful thought should be given to the audiologic rehabilitation of this patient group.     

Sensorineural hearing loss in Vogt-Koyanagi-Harada syndrome

BACKGROUND: Vogt-Koyanagi-Harada (VKH) syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Patients with VKH often report tinnitus and hearing loss, but these symptoms tend to be given secondary consideration because most undergo treatment with steroids to prevent blindness resulting from granulomatous uveitis, exudative retinal detachment, and optic nerve inflammation. METHODS/STUDY DESIGN: In the current retrospective review, 24 patients with this syndrome were screened for auditory system abnormalities. All patients denied history of noise exposure or ototoxic agent exposure. The age range of the patients was 13 to 42 years. RESULTS: Three patients reported tinnitus and two patients reported sudden hearing loss. One patient experienced vertigo and aural fullness. Eight of 24 patients had pure-tone thresholds greater than 25 dB hearing loss at two or more frequencies. Five of 24 of these patients experienced hearing loss outside of the 95% confidence interval for published age-matched control populations. There was sloping sensorineural hearing loss at 4 kHz and above in five of 24 patients. All eight patients with hearing loss experienced some degree of hearing loss at 4 kHz or above. Three patients had mild to moderate low-frequency sensorineural hearing loss. There were no tympanometric abnormalities suggestive of conductive involvement. Abnormal acoustic reflex decay was observed in one patient. CONCLUSIONS: We conclude that a significant number of patients with VKH experience sensorineural hearing loss and that every patient with VKH should undergo a review of systems for auditory abnormalities and referral for audiologic testing if symptomatic. It is possible that untreated patients may experience worse symptoms.     

Sensorineural hearing loss in Nigerian children with sickle cell disease

AIM: To study the prevalence and pattern of sensorineural hearing loss (SNHL) in Nigerian children with sickle cell disease (SCD). MATERIALS: Fifty-two children with SCD were assessed. Otologic examination and audiometric tests were performed. There were 36 males and 16 females. Their age ranged from 6 to 19 years. RESULT: Seven children had hearing impairment. After hemoglobin categorization, all the SCD patients were found to belong to the homozygous (HbSS) group. There were no HbSC nor other variants detected. Prevalence of SNHL was 13.4% in these patients but the general population prevalence rate found in a control group was 6.2%. CONCLUSION: SCD has many organ manifestations and complication among which is sensorineural hearing loss. Regular audiologic assessment, counselling and rehabilitation of these patients with hearing aids are recommended.     

Hearing loss in Wegener's granulomatosis.

OBJECTIVE: To describe the frequency, type, and clinical course of hearing loss in Wegener's granulomatosis and assess hearing loss as an indicator of disease activity. STUDY DESIGN, SETTING, AND PATIENTS: Retrospective cohort review of all patients with Wegener's granulomatosis seen in 1 year at an academic medical center. MAIN OUTCOME MEASURES: Hearing loss documented by pure-tone audiogram. RESULTS: Thirty-six patients were included in the analysis: 20 men and 16 women, with a mean age of 55.5 years (range, 22-87 yr); 30 (83%) were antineutrophil cytoplasmic autoantibodies-positive, and the mean disease duration was 47 months (range, 2-196 mo). Twenty patients (56%) had documented hearing loss: there were 17 (47%) cases of sensorineural hearing loss and 12 (33%) cases of conductive hearing loss. Seven of 12 cases of conductive hearing loss improved with immunosuppressive treatment of Wegener's granulomatosis, 2 worsened, and 3 remained stable. Of 17 cases of sensorineural hearing loss, 3 improved, 4 worsened, and 10 remained stable. Seven patients had hearing loss requiring amplification. Five of 35 (14%) patients had established hearing loss months to years before diagnosis of Wegener's granulomatosis. Hearing loss occurred both on initial presentation and with disease relapse. The rates of conductive hearing loss (38%) and sensorineural hearing loss (31%) were also high in the subset of patients 65 years of age or younger and without history of noise exposure. CONCLUSIONS: Both sensorineural hearing loss and conductive hearing loss are common in Wegener's granulomatosis, may result in significant morbidity, and may precede the diagnosis of Wegener's granulomatosis by years. Both types of hearing loss in patients with Wegener's granulomatosis may be used as an indicator of disease. These data suggest that it may be appropriate to obtain screening audiograms in all patients with newly diagnosed or relapsing Wegener's granulomatosis.     

Audiologic profiles of children with Kawasaki disease.

Kawasaki disease (KD) is an idiopathic vasculitis associated with systemic inflammation and profound immunoregulatory changes. Recent reports from Japan and the United States have documented the association of sensorineural hearing loss (SNHL) with acute KD. To further characterize the nature and prevalence of this complication, we prospectively evaluated the hearing of 40 consecutive patients with acute KD at a single institution. Standard audiometric procedures were used, including visual reinforcement audiometry and play audiometry. Auditory brainstem response (ABR) testing using clicks and tone pips (1000-4000 Hz) was performed in patients with abnormal or unreliable results on behavioral audiometry. Acoustic immittance measurements were obtained on all patients. Of the 23 males and 17 females (mean age 3.2 +/- 2.3 years, range 0.6-11.1 years), all but three were evaluated and treated with aspirin and intravenous gama globulin within 1 month of onset of fever. Seven children had test results suggesting sensorineural threshold shifts, 16 had normal hearing, and 14 had inconclusive hearing evaluations. Laboratory data in patients with hearing threshold shifts revealed significantly longer duration of fever (4.1 +/- 1.0 versus 1.9 +/- 0.5 days), and a tendency for higher temperatures and white blood cell counts at diagnosis compared to those with normal hearing. Results suggest that transient as well as persistent SNHL may be associated with the acute vasculitis of KD, and may be associated with laboratory markers indicating more severe systemic inflammation. Audiologic screening should be considered for all patients following KD.     

Prognostic factors of profound idiopathic sudden sensorineural hearing loss

Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1 %) and tinnitus (77.4 %). At the 2-month follow-up, 172 (29.8 %) patients showed some degree of hearing recovery, but only 21 (3.6 %) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.     

Otologic manifestations of malignant osteopetrosis.

OBJECTIVE: To determine the incidence of hearing loss and describe the neurotologic manifestations over time in a large series of patients with malignant osteopetrosis. STUDY DESIGN: Longitudinal study. SETTING: Tertiary care referral center. PATIENTS: Thirty-two patients, including 19 infants (< 1 yr of age at initial visit) and 13 children (aged 1-7.6 yr at initial visit), with autosomal recessive osteopetrosis were followed-up during a 10-year period from 1991 to 2001. The average length of follow-up was 2.5 years (range, 0-9.1 yr). INTERVENTIONS: Patients underwent annual otologic evaluations including clinical examination, audiologic evaluation (auditory brainstem response, pure-tone thresholds, speech discrimination scores, and tympanograms), and high-resolution computed tomography of the temporal bone. MAIN OUTCOME MEASURES: Incidence of hearing loss, otitis media, and facial paralysis. Serial changes in temporal bone anatomy by computed tomographic scan. RESULTS: Twenty-six percent of infants' ears demonstrated hearing loss during the first year of life, and 78% of children's ears demonstrated hearing loss during the study period. Of the children's ears with hearing loss, 100% had a conductive component and 26% had an additional sensorineural component (mixed hearing loss); VIIIth nerve conduction was normal in 100% of infants and 78% of children. Five patients (16%) had unilateral facial nerve palsy. Common temporal bone findings included thickening and sclerosis of the calvarium; poor pneumatization of the mastoid bone; and narrowing of the external auditory canal, eustachian tube, and internal auditory canal. CONCLUSION: Otologic manifestations are common in malignant osteopetrosis secondary to the formation of dense, brittle bone. Frequent findings include external auditory canal stenosis, otitis media, conductive and sensorineural hearing loss, and facial nerve paralysis.     

Craniofacial, temporal bone, and audiologic abnormalities in the spectrum of hemifacial microsomia

OBJECTIVES: To evaluate the clinical, audiologic, and temporal bone computed tomograpic findings in patients with hemifacial microsomia and to use the OMENS (each letter of the acronym indicates 1 of the following 5 dysmorphic manifestations: O, orbital asymmetry; M, mandibular hypoplasia; E, auricular deformity; N, nerve involvement; and S, soft tissue deficiency) grading system to assess possible correlations between the severity of dysmorphic features with the type of abnormalities in the temporal bone and with degree of hearing deficit. DESIGN: Retrospective study. SETTING: Tertiary care children's hospital. PATIENT: Forty patients with hemifacial microsomia. RESULT: Mandibular hypoplasia and auricular abnormalities were the most common clinical manifestations, present in 39 patients (97%) and 38 patients (95%), respectively. Conductive hearing loss was noted in 35 patients (86%) and sensorineural hearing loss in 4 patients (10%). Facial nerve weakness was present in 20 patients (50%). Twenty patients had unilateral aural atresia, 12 patients had unilateral aural stenosis, and 7 patients had bilateral anomalies. Moderate hypoplasia or atresia of the middle ear was noted in 36 patients (90%) and ossicles were malformed in 30 patients (75%). Hypoplasia of the oval window was the most common inner ear abnormality. CONCLUSIONS: Severity of craniofacial features (total OMENS score) significantly correlated with the degree of temporal bone abnormality, but no correlation was noted with the degree or type of hearing loss. We recommend the following: (1) use of the OMENS classification system for documentation and analysis of dysmorphic finding in hemifacial microsomia; (2) complete audiologic evaluation in all patients with hemifacial microsomia regardless of the type of craniofacial abnormalities; and (3) temporal bone computed tomography for further evaluation of hearing deficit.     

Otologic and audiologic features of Nager acrofacial dysostosis

OBJECTIVE: To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis. DESIGN: Retrospective case series. SETTING: Multidisciplinary clinic in a tertiary care children's hospital. SUBJECTS: Patients less than 18 years of age with Nager acrofacial dysostosis. METHODS: Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline. RESULTS: External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population. CONCLUSIONS: Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended.     

Congenital perilymphatic fistula: an overlooked diagnosis?

Congenital perilymphatic fistula (CPLF) often is the sole cause of sensorineural hearing loss in children that is both progressive and subtle and, therefore, can elude detection by physicians. A 3-year prospective study of 244 children with sensorineural hearing loss of unknown etiology demonstrated that at least 6% (15/244) had CPLF. Close audiologic monitoring and radiologic imaging of the temporal bone of children with sensorineural hearing loss are recommended to detect CPLF.     

Lyme disease--a reason for sudden sensorineural hearing loss and vestibular neuronitis?

BACKGROUND: Lyme disease has been described as one possible cause of sudden sensorineural hearing loss and vestibular neuronitis. The necessity of serological diagnosis and its therapeutic consequences have been discussed controversially. PATIENTS AND METHODS: 344 patients with acute sensorineural hearing loss and 66 patients with vestibular neuronitis were examined in retrospect. By means of ELISA (Enzygnost Borreliosis, Dade Behring Marburg) the specific prevalences of IgG- and IgM-antibodies against borrelia in serum were evaluated. The frequency of seroprevalences for both diseases were compared to those given in the literature. Neurootological findings of the seropositive patients were compared with those of seronegative and analysed statistically. RESULTS: 15.7 % of the patients with sudden sensorineural hearing loss had positive levels of IgG-antibodies. IgM-titers were elevated in 4.7 % of the patients. The seroprevalences for IgM and IgG were above those described by other investigators for the healthy population. Patients with positive IgM-antibodies showed more often low frequency hearing loss than IgG-positive patients. 18.2 % of the patients with neuronitis vestibularis had IgG- and 1.5 % IgM-antibodies against Borrelia. Whereas IgG occurred more often than known for the healthy population, IgM was within the limit for the healthy population. The seropositive group did not show any remarkable neurootological signs compared with the seronegative group. CONCLUSIONS: Because of the elevated seroprevalences Borrelia infections may be one possible but very rare cause of sudden sensorineural hearing loss and vestibular neuronitis. Low frequency hearing loss may be a sign for an infection with Borrelia as an etiological factor especially in combination with seropositive titers. In case of the presence of IgM-antibodies, patients may be treated with oral antibiotics (Doxycyclin, Cefuroxim). In patients with neuronitis vestibularis a neuroborreliosis should be excluded by means of lumbar puncture.     

Quantitative and qualitative follow-up outcomes from a preschool audiologic screening program: perspectives over a decade

PURPOSE: This investigation reports on quantitative and qualitative follow-up information obtained from a preschool audiologic screening program covering a 10-year period (1995 to 2004). METHOD: The audiologic screening consisted of a hearing (pure tone) and tympanometry screening. A total of 34,979 children, 3 to 5 years of age, were screened. RESULTS: Eighteen percent (6,337) of the children were referred for further hearing and/or medical ear evaluation. Of 1,421 follow-up responses received, 93% complied with the follow-up recommendations while 7% did not. Of 1,316 children in the follow-up group, outer and/or middle ear disorder in one or both ears was medically confirmed for 37%. Unilateral or bilateral hearing loss was diagnosed in 18% as conductive (12%), sensorineural (1%), mixed (0.4%), or unspecified (5%). Overall, hearing loss and/or otologic disorder was confirmed in 49% of the follow-up group, suggesting a prevalence of 1.8% in a preschool-age population. A small (n = 32) sample of unsolicited comments indicated that physicians most influenced noncompliance with hearing evaluation follow-up. CONCLUSIONS: The quantitative hearing and otologic follow-up outcome data affirm the importance of audiologic screening in the preschool population. Qualitative data suggest that some physicians may not be advocating appropriate screening follow-up services.     

Facioscapulohumeral muscular dystrophy and accompanying hearing loss

Facioscapulohumeral (FSH) muscular dystrophy (MD) is an autosomal dominant condition marked by muscle weakness primarily in the face, shoulder girdle, and legs. Intelligence and life span are normal and the spectrum of disability is broad. Flaccid dysarthria results from the facial muscle paralysis. A pair of siblings had FSH MD that was accompanied by the unusual finding of sensorineural hearing loss. Speech and audiologic aspects are presented and etiologic explanations are suggested. Careful audiologic evaluation of all patients with FSH MD is advised so that sensorineural hearing loss can be ruled out.