Objectives/Hypothesis Cleidocranial dysplasia is an autosomal dominant skeletal syndrome characterized by open skull sutures and clavicular hypoplasia or aplasia. It results from mutations in the transcription factor CBFA1 required for osteoblast differentiation and normal bone formation. Therefore, mutations in CBFA1 would be expected to cause conductive or sensorineural hearing loss, or both. The objective of the study was to evaluate the auditory function and head and neck manifestations of patients with cleidocranial dysplasia. Study Design Case series. Methods Patients with cleidocranial dysplasia who were identified from the case records of the Craniofacial Anomalies Center at the University of California San Francisco (San Francisco, CA) gave medical and developmental history, underwent a general physical examination and a thorough head and neck examination, and completed a comprehensive audiological evaluation. Results Four families with eight affected individuals in all and one sporadic case were studied. The nine patients showed highly variable expression of abnormalities. Head and neck examination demonstrated abnormal cranial sutures, broad nasal root, clavicular hypoplasia or aplasia, and abnormally high arched palate. Although seven of nine patients showed eustachian tube dysfunction, recurrent otitis media in childhood requiring surgical intervention was not common. Hearing loss was present in three of nine patients (33%). One patient had a 40‐dB mixed hearing loss, the second had a 25‐dB low‐frequency conductive hearing loss, and the third had a 45‐dB high‐frequency sensorineural hearing loss. Conclusion In patients with cleidocranial dysplasia, eustachian tube dysfunction, conductive hearing loss, and sensorineural hearing loss are common because of structural and functional changes of the temporal bone and palate. Therefore, patients with cleidocranial dysplasia should be evaluated routinely by an otolaryngologist and undergo complete audiological testing.

Differences in performance were evaluated between the Widex Senso C8 (omnidirectional) and C9 (directional) hearing aids and analog hearing aids currently worn by 40 subjects with hearing loss. Subjects were fit with the C8 and C9 using the manufacturer's recommended procedure. Differences in performance between the C8 and C9 and the subjects' own hearing aids were assessed using the Speech Perception in Noise (SPIN) test administered at +7, 0, and -7 dB signal-to-noise ratio (SNR) with the noise fixed at 65 and 75 dB SPL. Also, a questionnaire was completed assessing differences in preference between the C9 and the subjects' own hearing aids. The major finding was the presence of a significant advantage of the C9 relative to the C8 and the subjects' own hearing aids at each experimental condition. The magnitude of the advantage provided by the C9 increased as the SNR became more difficult. However, significant differences were not present between the C8 and the subjects' own hearing aids at any experimental condition. The questionnaire revealed a statistically significant preference for the C9 in comparison to the subjects' own hearing aids. Abbreviations: ANSI = American National Standards Institute, BTE = behind the ear, CIC = completely in the canal, DSP = digital signal processing, FBR = front-to-back ratio, HP = high predictability, ITE = in the ear, LP = low predictability, PI = performance intensity, SNR = signal-to-noise ratio, SPIN = Speech Perception in Noise

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