Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve.

OBJECTIVE: The aim of our study is to present the results of 11 children where auditory brainstem implantation (ABI) was successfully performed to restore hearing. STUDY DESIGN: Case presentation. This study was conducted at the departments of Otolaryngology and Neurosurgery at Hacettepe University Ankara, Turkey. PATIENTS: Between July 2006 and April 2008, 11 prelingual (30-56 mo) deaf children with several cochlear malformations had ABI. INTERVENTION: All patients were programmed and were enrolled in auditory verbal therapy sessions and family counseling programs at Hacettepe Auditory Verbal Center. The evaluation was performed at preimplant and again 1, 3, 6, 9, and 12 months post-switch on. MAIN OUTCOME MEASURES: The main test components composing this test battery were Ling 6 Sound Detection-Identification Test, Word Identification Test in Turkish, Meaningful Auditory Integration Scale, and Meaningful Use of Speech Scale. RESULTS: Successful brainstem implantations were performed in all patients with retrosigmoid approach. Six children gained basic audiologic functions and were able to recognize and discriminate sounds, and many could identify environmental sounds such as a doorbell and telephone ring by the third month of ABI. Improvement in mean performance on Meaningful Auditory Integration Scale is apparent for all ABI children. Improvement in Meaningful Use of Speech Scale scores in 2 patients, demonstrating that the child using its own voice for speech performance, was observed between the baseline and 12th month. First, 5 children were able to identify Ling's 6 sound by the end of 2 to 6 months, and 2 of them also started to identify words due to their pattern differences and multisyllabic word identification by 6 to 9 months. Two children with Attention Deficit Hyperactivity Disorder have made slower progress than the other children with ABIs. CONCLUSION: Our preliminary results show that there is adequate contribution of brainstem implants in the development of auditory-verbal skills. Additional handicaps slow the progress of the prelingually deaf children.

Unusual spindle cell tumor of thyroid (SETTLE).

Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare tumor seen mostly in children and adolescents. It is presumed that it arises from ectopic thymus or remnants of branchial pouches that differentiate along the thymic line. Unusual spindle cell-predominant histology makes this entity difficult to recognize. The clinical course is not exactly known due to the small number of reported cases but metastasis has been documented during initial presentation. We report a case of SETTLE in the neck where preoperative clinical and radiological impression was that of a mass in the left submandibular gland area. However, during the operation it was felt by the surgeon that the mass was in continuity with the left thyroid lobe.

Bony sheathed chorda tympani: a unique case of incudomalleolar fixation.

OBJECTIVE: To describe a unilateral conductive hearing loss caused by restriction of the movements of malleus and incus due to chorda tympani in a bony sheath. PATIENT: A 23-year-old man with a stable mild conductive hearing loss in the left ear and mild sensorineural hearing loss in the right ear. INTERVENTION: An exploratory tympanotomy was performed. MAIN OUTCOME MEASURE: Immobility of the ossicles due to a bony structure, which was found to be chorda tympani encircled in a bony sheath. Ossicular system regained its mobility with the severance of the chorda tympani. RESULTS: Hearing was improved both subjectively and audiographically. CONCLUSION: This is the second case of chorda tympani with a bony sheath and the first case of this pathologic finding affecting both malleus and incus. Bony sheathed chorda tympani is considered to be a congenital anomaly of the chorda tympani that causes incudomalleolar fixation and behaves as posterior bony bars.

Scanning electron microscopy of ciliae and saccharine test for ciliary function in septal deviations.

OBJECTIVE: To investigate the difference in mucociliary clearance and surface mucosal structure of the nasal septum and lateral nasal wall in patients with and without septal deviation. METHOD: The saccharine-dye test was used to measure the mucociliary clearance time in both nasal cavities of 20 patients with nasal septal deviation (study group) and was compared with that of 30 patients without septal deviation (control group). Bilateral septal and lateral nasal wall mucosal biopsies were taken from the study group during septoplasty, and unilateral biopsies were taken from 10 of the control group. These biopsies were studied under the scanning electron microscope. RESULTS: In the study group, mucociliary clearance on the side opposite the septal deviation was significantly slower than on the other side. Mucociliary clearance on both sides of the deviated septum of the study group was significantly slower than clearance in the control group. There was no statistically significant difference in the distribution of mucosal cilia of the cavities on either side of the deviated septum in the study group, nor between the distribution in the study group and controls. CONCLUSION: Patients with septal deviation display no change in mucosal surface anatomy but have decreased mucociliary activity on both sides of the deviation, the least activity being on the side opposite the deviation.

Prognostic significance of Langerhans cell infiltration in cancer of the larynx.

OBJECTIVE: Determine the effect on prognosis of Langerhans cell infiltration in cancer of the larynx. STUDY DESIGN AND SETTING: Retrospective review of 72 surgically treated patients with T1-3 lesions. The streptavidin-biotin method to determine Langerhans cell infiltration, which was graded as 1+, 2+, and 3+. RESULTS: A higher degree of Langerhans cell infiltration was significantly associated with less cervical lymph node metastasis, longer disease-free survival, less locoregional recurrence, and less clinical N-positivity ( P < 0.05). According to multivariant analysis, Langerhans cell infiltration was independently related to disease-free survival and recurrence ( P < 0.05). CONCLUSIONS: Langerhans cell infiltration is prognostically important in cancer of the larynx. More intense infiltration is significantly related to prolonged disease-free survival, less locoregional recurrence, less cervical lymph node metastasis, and less clinical N-positivity. SIGNIFICANCE: Langerhans cell infiltration may be determined on a biopsy specimen and this information may be useful in deciding about elective neck dissection. Patients with mild infiltration may have a higher risk of locoregional recurrence, shorter disease-free survival, and therefore they suggest a poor prognosis.

Magnetic resonance imaging versus computed tomography in pre-operative evaluation of cochlear implant candidates with congenital hearing loss.

Recent reports indicate that the cochlear nerve may be absent in some cases of congenital sensorineural hearing loss. The aim of this prospective study was to determine the incidence of cochlear nerve anomaly in cochlear implant candidates with congenital hearing loss using magnetic resonance imaging (MRI). Twenty-seven patients with congenital profound bilateral sensorineural hearing loss who were being evaluated for the cochlear implant procedure were studied. These patients had high-resolution computerized tomography (CT), through the petrous bone in axial sections. MRI examinations consisted of T1 and turbo spin echo (TSE) T2-weighted 3 mm axial images, and additional 3D Fourier Transform T2-weighted TSE sequences obtained on three different planes (axial, perpendicular and parallel to the internal auditory canal (IAC) i.e. oblique sagittal and coronal, respectively) for the purpose of cochlear nerve demonstration. Results showed that all of the 14 patients with normal CT of the temporal bone, had four distinct nerves in the distal part of the IAC on TSE-MRI. Thirteen patients demonstrated various bony malformations of the cochleovestibular system on CT. MRI revealed the absence of the cochleovestibular nerve in four patients where the IAC was very narrow or completely absent on CT. One patient with severe Mondini malformation who had an enlarged IAC demonstrated an isolated absent cochlear nerve.