Fetal alcohol syndrome (FAS) is characterized by congenital anomalies traditionally associated with hearing disorders. The present study sought to (a) evaluate possible central hearing loss; (b) verify and extend previous observations on sensorineural and conductive hearing losses; (c) evaluate possible vestibular disorders; (d) examine the relationships between hearing, speech, language, vestibular, and dentofacial disorders in FAS patients; and (e) evaluate the influence of patient age, race, and gender on the expression of these morbidities. A biracial group of 22 FAS patients (aged 3 to 26 years) were evaluated by standard hearing, speech, language, and vestibular tests. Dentofacial and other malformations were also assessed. Of the 22 FAS patients, 17 (77%) had intermittent conductive hearing loss due to recurrent serous otitis media that persisted from early childhood into adulthood, whereas 6 (27%) had sensorineural hearing loss in addition to the conductive hearing loss. Among the 12 patients tested for central hearing function, all (100%) were significantly impaired. Among the patients tested for speech and language ability, 18 of 20 (90%) had speech pathology, 16 of 21 (76%) had expressive language deficits, and 18 of 22 (82%) had receptive language deficits. Hearing, speech, and language deficits were not influenced by age, race, or gender. On the vestibular tests, all performed within normal limits with the possible exception of one child (n = 6). High incidences of dentofacial, temporomandibular joint, ocular, cardiac, and skeletal disorders were observed. Race and gender tended to influence dental malocclusion class. Two subjects exhibited autistic tendencies. In conclusion, new and important findings included a high prevalence of sensorineural, conductive, and central hearing deficits, the persistence of otitis proneness into adulthood, the existence of temporomandibular joint disorders, and the possible influence of gender or race on dental malocclusions. Such disorders can contribute to the learning, behavioral, and emotional difficulties seen in FAS patients and warrant early, aggressive intervention.

Fetal alcohol syndrome (FAS) is characterized by congenital anomalies traditionally associated with hearing disorders. The present study sought to (a) evaluate possible central hearing loss; (b) verify and extend previous observations on sensorineural and conductive hearing losses; (c) evaluate possible vestibular disorders; (d) examine the relationships between hearing, speech, language, vestibular, and dentofacial disorders in FAS patients; and (e) evaluate the influence of patient age, race, and gender on the expression of these morbidities. A biracial group of 22 FAS patients (aged 3 to 26 years) were evaluated by standard hearing, speech, language, and vestibular tests. Dentofacial and other malformations were also assessed. Of the 22 FAS patients, 17 (77%) had intermittent conductive hearing loss due to recurrent serous otitis media that persisted from early childhood into adulthood, whereas 6 (27%) had sensorineural hearing loss in addition to the conductive hearing loss. Among the 12 patients tested for central hearing function, all (100%) were significantly impaired. Among the patients tested for speech and language ability, 18 of 20 (90%) had speech pathology, 16 of 21 (76%) had expressive language deficits, and 18 of 22 (82%) had receptive language deficits. Hearing, speech, and language deficits were not influenced by age, race, or gender. On the vestibular tests, all performed within normal limits with the possible exception of one child (n = 6). High incidences of dentofacial, temporomandibular joint, ocular, cardiac, and skeletal disorders were observed. Race and gender tended to influence dental malocclusion class. Two subjects exhibited autistic tendencies. In conclusion, new and important findings included a high prevalence of sensorineural, conductive, and central hearing deficits, the persistence of otitis proneness into adulthood, the existence of temporomandibular joint disorders, and the possible influence of gender or race on dental malocclusions. Such disorders can contribute to the learning, behavioral, and emotional difficulties seen in FAS patients and warrant early, aggressive intervention.

These previously undocumented auditory brainstem response abnormalities reflect abnormal neural transmission, which could cause peripheral and central auditory processing disorders. We speculate that the major pathogenic basis of the I-to-III interpeak latency and wave II abnormalities is compression of the auditory nerve as it passes through the internal auditory meatus and posterior fossa, which would explain the auditory nerve hearing loss, tinnitus, and vertigo that affect these children. Awareness of these abnormalities could lead to important advancements in the auditory and neurosurgical assessment and management of this overlooked patient group. We provide recommendations for the improved assessment and management of these patients. In particular, we recommend that auditory brainstem response diagnostics become standard clinical care for this patient group as the best way to detect auditory nerve compression.

Ninety-eight cleft palate subjects were studied to ascertain the eventual adolescent and adult hearing. There were 72 complete clefts of lip and palate and 26 clefts of the palate alone. The incidence or side of ear disease did not appear to be influenced by this type of cleft. Other systemic anomalies were present in 11 cases with congenital middle ear abnormalities in 3 and sensory neural hearing loss in 8 subjects. Progressive improvement in incidence of hearing loss from 94% at 4 years to 42% at 20 years was noted with the strict definition of hearing loss as 10 dB conductive loss at any one frequency. 10% of ears were left with permanent structural changes in the middle ear. In only 2 patients was there a bilateral hearing loss of 20 dB or more through the speech range.