Malleus bar: an unusual ossicular abnormality in the setting of congenital aural atresia.

OBJECTIVES: Malleus bar is a rare cause of conductive hearing loss and has yet to be described in the setting of congenital aural atresia. This study aimed to define the anomaly and review the surgical management and outcomes of patients found to have a malleus bar in the setting of congenital aural atresia. STUDY DESIGN: Retrospective case review of patients with malleus bar and congenital aural atresia. SETTING: Tertiary otologic referral center. PATIENTS: Patients found to have a malleus bar at the time of surgery for congenital aural atresia. METHODS: Charts of patients who underwent surgery for congenital aural atresia repair with a malleus bar were reviewed for demographic data, preoperative Jahrsdoerfer score, surgical findings, and audiometric data. RESULTS: Seven subjects (8 ears) were identified to have a malleus bar and congenital aural atresia. In all 8 ears, the malleus bar proved to be a second point of attachment of the ossicular chain in addition to the atretic plate. The chorda tympani nerve was found in the bony bar in 4 patients. Closure of the air-bone gap within 35 dB hearing level (HL) was achieved in 83% of patients. All patients showed improved speech reception thresholds and stable bone-conduction thresholds. All but 1 patient had stable or improved word recognition scores. CONCLUSION: Malleus bar in the setting of congenital aural atresia represents a rare combination of causes of congenital conductive hearing loss. Patients with these 2 abnormalities obtain good short-term results with surgical repair of congenital aural atresia.

Reinforcement of cell junctions correlates with the absence of hair cell regeneration in mammals and its occurrence in birds.

Debilitating hearing and balance deficits often arise through damage to the inner ear's hair cells. For humans and other mammals, such deficits are permanent, but nonmammalian vertebrates can quickly recover hearing and balance through their innate capacity to regenerate hair cells. The biological basis for this difference has remained unknown, but recent investigations in wounded balance epithelia have shown that proliferation follows cellular spreading at sites of injury. As mammalian ears mature during the first weeks after birth, the capacity for spreading and proliferation declines sharply. In seeking the basis for those declines, we investigated the circumferential bands of F-actin that bracket the apical junctions between supporting cells in the gravity-sensitive utricle. We found that those bands grow much thicker as mice and humans mature postnatally, whereas their counterparts in chickens remain thin from hatching through adulthood. When we cultured utricular epithelia from chickens, we found that cellular spreading and proliferation both continued at high levels, even in the epithelia from adults. In contrast, the substantial reinforcement of the circumferential F-actin bands in mammals coincides with the steep declines in cell spreading and production established in earlier experiments. We propose that the presence of thin F-actin bands at the junctions between avian supporting cells may contribute to the lifelong persistence of their capacity for shape change, cell proliferation, and hair cell replacement and that the postnatal reinforcement of the F-actin bands in maturing humans and other mammals may have an important role in limiting hair cell regeneration.

Auricular ossification resulting in external auditory canal stenosis.

We report the case of a 49-year-old man who presented with left aural fullness, hearing loss, and a stenotic left external auditory meatus. Physical examination and computed tomography revealed the presence of an idiopathic ossification of the cartilaginous auricle and external auditory canal. The patient was successfully treated with meatoplasty. Pathologic examination of the removed specimen revealed osseous metaplasia.

Malignant otitis externa.

Malignant otitis externa is an invasive, potentially life-threatening infection of the external ear and skull base that requires urgent diagnosis and treatment. It affects immunocompromised individuals, particularly those who have diabetes. The most common causative agent remains Pseudomonas aeruginosa. Definitive diagnosis is frequently elusive, requiring a high index of suspicion, various laboratory and imaging modalities, and histologic exclusion of malignancy. Long-term oral antipseudomonal agents have proven effective; however, pseudomonal antibiotic resistance patterns have emerged and therefore other bacterial and fungal causative agents must be considered. Adjunctive therapies, such as aggressive debridement and hyperbaric oxygen therapy, are reserved for extensive or unresponsive cases.

3 Tesla delayed contrast magnetic resonance imaging evaluation of Ménière's disease.

OBJECTIVE: To determine whether 3 Tesla (T) magnetic resonance imaging (MRI) with delayed contrast imaging has sufficient anatomic resolution to image the intracochlear fluid spaces (i.e., the scala tympani, scala media, and scala vestibuli) of the inner ear and identify endolymphatic hydrops in vivo. STUDY DESIGN: Four normal subjects underwent 3T MRI scanning after the administration of gadodiamide intravenous (IV) contrast. MRI region of interest signal intensity was used to determine the diffusion of gadodiamide into the perilymphatic fluid spaces over time. Seven subjects with unilateral Ménière's disease underwent 3T MRI 4 hours after administration of gadodiamide IV contrast agent. RESULTS: All four normal subjects demonstrated perilymphatic enhancement by 4 hours postinjection. Loss of definition of the membranous cochlea was noted in five of the seven subjects with Ménière's disease. Precise definition of the scala media could not be achieved with this imaging protocol. CONCLUSION: Delayed contrast imaging of the inner ear with 3T MRI revealed in vivo changes of the membranous labyrinth consistent with unilateral Ménière's disease.

Underlay tympanoplasty with laser tissue welding.

We investigated the feasibility of using laser tissue welding techniques to perform transcanal underlay tympanoplasty. We used 10 temporal bones obtained from human cadavers. After creating a subtotal tympanic membrane perforation, we introduced harvested periosteum through the perforation and used laser tissue welding to secure the periosteum graft in place in an underlay fashion. The procedure was performed via a transcanal approach and did not require middle ear packing. Immediately after the graft had been placed, we qualitatively tested its integrity with a blunt probe. The graft was as strong as the native cadaver tympanic membrane in all 10 cases. We conclude that laser transcanal underlay tympanoplasty is a feasible and effective method of repairing a tympanic membrane. The ultimate goal is to develop a technique that will allow physicians to routinely perform underlay tympanoplasty on moderately sized perforations in an office setting.

Minimal access surgery for the Symphonix/Med-El Vibrant Soundbridge middle ear hearing implant.

OBJECTIVE: To develop a minimal access approach for implantation of the Vibrant Soundbridge middle ear hearing implant. This approach ideally uses the smallest skin incision possible, minimal or no hair shave, and the least possible amount of tissue and bone manipulation. This will facilitate the acceptability of the procedure to the general community and reduce the flap-related complication rate. The procedure is similar to the minimal access approach described for cochlear implantation. STUDY DESIGN: Eight patients with various degrees of sensorineural hearing loss and one with a mixed hearing loss who met implant criteria for the Vibrant Soundbridge middle ear hearing implant received the device over a 42-month period. The first two patients underwent the traditional implant procedure with postauricular hair shave, postauricular S-shaped incision, and implant receiver suture fixation to the temporal bone. The following seven consecutive patients received a progressively smaller C-shaped postauricular skin incision, no hair shave, retrograde skull drilling for the implant seat, and no implant suture fixation until the technique closely approximated the minimal access cochlear implant procedure. Postoperative performance of the Soundbridge/Vibrant Med-El was evaluated through audiology testing and subjective patient reports. SETTING: Private neurotology clinic and tertiary care teaching hospital. RESULTS: The technique was feasible in all patients. Follow-up for the minimal access group ranged from 3 years to 5 months. There were no complications related to the approach, and all patients were satisfied users of the implant. The lack of hair shave and small incision size was greatly appreciated and warmly endorsed by the patients. CONCLUSION: The Vibrant Soundbridge/Vibrant Med-El can be safely implanted using the minimal access method that has been popularized for cochlear implant surgery. A large incision, extensive hair shave, risk of flap necrosis, and possibility of unsightly scar may deter patients from pursuing the potential benefits of implanted hearing technology. The technique may make the device more accessible to individuals who have concerns regarding cosmetics and potential flap complications.