Bilateral External Auditory Canal Atresia Research Articles

Cleft Palate, Bilateral External Auditory Canal Atresia, and Other Midline Defects Associated With Diamond-Blackfan Anemia

Diamond-Blackfan anemia (DBA) is associated with congenital anomalies especially of the midline. When present, facial anomalies are reminiscent of Treacher-Collins syndrome, and both DBA and Treacher-Collins syndrome are disorders of ribosomal biogenesis. Herein, we describe a female infant with multiple midline defects associated with DBA and reaffirm the absence of RPS-19 mutations in DBA patients with facial anomalies.

Bone-conducted vestibular evoked myogenic potentials in patients with congenital atresia of the external auditory canal

Objective: The purpose of this study was to determine whether vestibular evoked myogenic potentials from the sternocleidomastoid muscle in response to bone-conducted clicks and short tone-bursts can be used to assess vestibular apparatus function in patients with conductive hearing problems, particularly bilateral external auditory canal atresia. Design: Evoked-potential responses to bone-conducted auditory stimuli were recorded from the sternocleidomastoid muscle of 15 patients (11 male and four female, aged 4–20 years) with congenital bilateral atresia of the external auditory canal, with or without the middle ear anomalies. Setting: This study was conducted in the outpatient clinic of the Tokyo University Hospital, Department of Otolaryngology, University of Tokyo. Intervention: Diagnostic. Outcome measures: Bone-conducted vestibular evoked myogenic potentials in response to clicks and short tone-bursts were recorded with surface electrodes over both sternocleidomastoids in each patient. Results: In all patients, bone-conducted clicks and short tone-bursts evoked larger biphasic responses from the sternocleidomastoid ipsilateral to the stimulated ear. Short tone-bursts evoked vestibular evoked myogenic potentials with higher amplitude and better waveform morphology than clicks at the same subjective intensity. Conclusion: Loud auditory stimuli delivered by bone conduction can evoke myogenic potentials from the sternocleidomastoid. This method is a noninvasive, rapid, and convenient test for investigating the vestibular system function in patients with bilateral external auditory canal atresia, with or without the middle ear anomalies.

Audiologic Management of Bilateral External Auditory Canal Atresia with the Bone Conducting Implantable Hearing Device

Audiologic Management of Bilateral External Auditory Canal Atresia with the Bone Conducting Implantable Hearing Device

Audiologic Management of Bilateral External Auditory Canal Atresia With the Bone Conducting Implantable Hearing Device

The hearing impairment associated with congenital external auditory canal atresia has been managed with early bone conduction hearing aid placement and surgical reconstruction in selected patients. However, many patients do not wear a bone conduction hearing aid because of physical or social considerations and surgical reconstruction of the external auditory canal and middle ear may be difficult or contraindicated. This report details the use of implantable bone conducting hearing devices in five children with bilateral external auditory canal atresia. Each patient had bilateral conductive hearing impairment with normal bone conduction thresholds. Four of the five patients had associated craniofacial anomalies including three cases of microtia. The average preoperative sound field speech reception threshold improved from 63 dB to 13 dB with the implant. Patients experienced a definite preference for the implanted hearing device over the bone conduction hearing aid.