Attitudes of the UK ear, nose and throat clinical community to the future potential use of stem cell therapies to treat deafness.

Aims: Hearing loss is commonly due to the degeneration and death of hair cells and their associated spiral ganglion neurons. A total of 250 million people are affected worldwide. Stem cell treatments offer new and powerful strategies to enable recovery from hearing loss.

Cochlear implantations in children with Waardenburg syndrome: an electrophysiological and psychophysical review.

Waardenburg syndrome presents with dystopia canthorum, pigmentary abnormalities of hair, iris and skin (often a white forelock and heterochromia iridis) and sensorineural deafness. The authors review the electrophysiological and psychophysical findings of implanted children with Waardenburg syndrome at the Sydney Cochlear Implant Centre. Twenty children with Waardenburg syndrome received cochlear implants between 1985 and 2001.

Trans-tympanic electric auditory brainstem response (TT-EABR): the importance of the positioning of the stimulating electrode.

Trans-tympanic electric auditory brainstem response (TT-EABR) is an established pre-operative investigation in cochlear implantation surgery. Various techniques have been employed to obtain electrical responses but there has been no universal agreement on the exact positioning of the stimulating electrode on the medial wall of the mesotympanum. The authors investigate the relationship of the positioning of the electrode and the brainstem response.

Microsurgical access for cell injection into the mammalian cochlea.

The potential use of stem cells to repair hearing loss requires surgical access to the cochlea. Here we describe a microsurgical technique for cell injection into the mouse cochlea. Green fluorescent cells (ZsGreen-MCF10A cells) were successfully injected via a lateral wall cochleostomy into the scala media, scala tympani and scala vestibuli compartments of the cochlea.

Displacement of electrodes of a cochlear implant into the vestibular system: intra- and postoperative electrophysiological analyses.

A cochlear implant device is normally inserted into the scala tympani via a posterior tympanotomy and cochleostomy. There has been no previous report of displacement of the array into the vestibular part of the labyrinth. The authors present and discuss the audiological and electrophysiological measurements of a case in which part of the array herniated through into the vestibule.

Hush puppy: a new mouse mutant with pinna, ossicle, and inner ear defects.

Objectives/hypothesis: Deafness can be associated with abnormalities of the pinna, ossicles, and cochlea. The authors studied a newly generated mouse mutant with pinna defects and asked whether these defects are associated with peripheral auditory or facial skeletal abnormalities, or both. Furthermore, the authors investigated where the mutation responsible for these defects was located in the mouse genome.

Characterization of a new mouse mutant, flouncer, with a balance defect and inner ear malformation.

Hypothesis: Balance anomalies are often associated with abnormalities of the vestibular part of the inner ear. We studied a newly generated mouse mutant with balance defects and asked whether its behavioral anomalies were associated with inner ear defects. Furthermore, we asked whether the mutation responsible for the defects was located in the same region of mouse chromosome 4 as several other mouse mutations that we have previously described.