Tomo Yamaguchi

Surgical Strategy of Cochlear Implantation in Patients with Chronic Middle Ear Disease

We report 10 postlingually deafened adults in whom the electrophysical criteria for cochlear implant were fulfilled, except that they showed the following unfavorable middle ear lesions: otitis media with effusion, chronic perforative otitis media, cholesteatoma and previous radical ear operation. Staged operations for cochlear implant were performed in 8 cases, and 2 patients who had undergone radical ear operation had a single-stage operation. As a first step, one of the following was performed in each patient as surgically indicated: myringoplasty with or without mastoidectomy, mastoidectomy with reconstruction of the posterior wall of the external canal, mastoidectomy with the insertion of a ventilation tube, radical mastoidectomy or surgical cleansing of the radical cavity. From 6 months to 2.5 years after the first operation, the actual cochlear implant was performed in the second or third stage. There was no major complication as a result of electrode insertion into the cochlea and the results of speech perception in these cases were not different from those in patients with normal middle ears. In our experience, it was considered that the staged operations would enable successful cochlear implants in selected patients with pathological middle ear lesions even if they had previously been diagnosed as contraindicated for this procedure. In a case with radical ear cavity a single-stage operation could be performed when there was no cavity problem.

Cochlear Implantation in a Patient with Mitochondrial Disease-Kearns-Sayre Syndrome: A Case Report

Kearns-Sayre syndrome (KSS) is one of a mitochondrial disease characterized by progressive eye disorders, such as pigmentary retinopathy, and complete heart blockage. A recent study revealed that the etiology of this syndrome involves sporadic large scale rearrangements of mitochondrial DNA. Other neurologic symptoms are frequently associated with this syndrome, and hearing loss is the other main symptom. At the age of 37 years, this female patient began to suffer progressive hearing loss, and eventually became completely deaf. MRI imaging showed diffuse, abnormally high signals in the brain white matter, in T2-weighted images. Further neurological examination revealed eye abnormalities, and a genetic analysis confirmed a diagnosis of KSS. In this case, no sensory agnosia or other focal signs of disorder in the brain cortex were found, and the electrophysical criteria for cochlear implant were fulfilled. Cochlear implantation was performed on May 19, 1995. Post-operatively, the patient underwent vowel and consonant recognition tests. The patients scored well with an accuracy of 75% for vowels and 36% for consonants. The accuracy of vowel and consonant recognition with lip reading was elevated to 100% and 72%, respectively. Sound recognition in this patient seemed to be quite high compared to that reported in other implanted patients. The excellent results obtained in this KSS patient suggest pure neural deafness, and raise the possibility of cochlear involvement in this condition.

Relation between Vestibular Function and Speech Recognition in Postlingually Deafened Adults with Cochlear Implantation

Thirty-one postlingually deafened adults with 22-channel cochlear implants were evaluated preoperatively using vestibular function tests, the caloric test and stabilometry. The results of caloric response were classified into two diagnostic groups: normo- or hyporeflexia and areflexia. Stabilometric recordings were made using a computerized static posturographic platform. Between the two categories of caloric response, there was a significant difference in consonant recognition, but the recognition of vowels showed no significant difference. The results of the distance of body sway in stabilometry, but not of the area, correlated to the results of consonant recognition in speech perception tests. The degree of residual vestibular function seems to be one of the indicators influencing postoperative speech recognition by cochlear implant patients.

Evaluation of Acute Hearing Loss in Three Cases of Mitochondrial Encephalomyopathy.

Mitochondrial encephalomyopathy is a multisystem disease, affecting predominantly the CNS and skeletal muscles, and is caused by defects in mitochondrial function. There are three distinctive syndromes: KSS (Kearns-Sayre syndrome), MELAS (mitochondrial myopathy, encephalomyopathy, lactic acidosis and stroke-like episodes), and MERRF (myoclonus epilepsy with ragged-red fibers).Hearing impairment is one of the symptoms of mitochondrial encephalomyopathy. There have been several reports of progressive sensorineural hearing loss in children and adults with this disease. Acute hearing loss has not however, been previously reported. Two patients with KSS and one patient with MELAS, who showed sudden onset hearing loss, are described in this paper.

Two Cases of B cell Lymphoma with Skin Infiltration

Non-Hodgkins lymphoma usually occurs in the head and neck regions. It is commonly known. that T cell lymphoma infiltrates the skin, while B cell lymphoma is rarely associated with skin infiltration. We report two cases of B cell lymphoma with skin infiltration. The first case was a 58-yearold female with swelling of the right tonsil. She was treated with chemotherapy (2 cycles VEPA) and radiotherapy. The second case was an 82-year-old female with swelling of the left cheek, who was treated with radiotherapy. Histopathological features of these two cases showed diffuse large cell types (LSGs classification). Despite treatment, both patients died from B cell lymphoma infiltration of the skin.