Most patients with a Zenker's diverticulum are good candidates for endoscopic management. In our series, 84% of those who underwent endoscopic treatment ultimately achieved relief of symptoms. The patient morbidity is minimal. A large diverticulum with redundant mucosa is a risk factor for recurrence after endoscopic treatment.
The BETA2/NeuroD1 null mouse has cochlear dysplasia. Its cochlear duct is shorter than normal, there is a lack of spiral ganglion neurons, and there is hair cell disorganization. We measured vertical movements of the tectorial membrane at acoustic frequencies in excised cochleae in response to mechanical stimulation of the stapes using laser doppler vibrometry. While tuning curve sharpness was similar between wild-type, heterozygotes, and null mice in the base, null mutants had broader tuning in the apex. At both the base and the apex, null mice had less phase lag accumulation with increasing stimulus frequency than wild-type or heterozygote mice. In vivo studies demonstrated that the null mouse lacked distortion product otoacoustic emissions, and the cochlear microphonic and endocochlear potential were found to be severely reduced. Electrically evoked otoacoustic emissions could be elicited, although the amplitudes were lower than those of wild-type mice. Cochlear cross-sections revealed an incomplete partition malformation, with fenestrations within the modiolus that connected the cochlear turns. Outer hair cells from null mice demonstrated the normal pattern of prestin expression within their lateral walls and normal FM 1-43 dye entry.Overall, these data demonstrate that while tonotopicity can exist with cochlear dysplasia, traveling wave propagation is abnormally fast. Additionally, the presence of electrically evoked otoacoustic emissions suggests that outer hair cell reverse transduction is present, although the acoustic response is shaped by the alterations in cochlear mechanics.
Multiple-use atomizers that contain phenylephrine and tetracaine solutions with bacteriostatic preservatives in an outpatient otolaryngology clinic do not yield significant bacterial growth and, when used as described, do not pose an infectious risk of bacterial transmission between patients.
The circumferential petrosectomy is a potential treatment option when medical treatment fails in patients with petrositis or cranial base osteomyelitis. It permits maximal temporal bone debridement while preserving hearing and facial nerve integrity in these life-threatening disease processes.
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.
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