Totally endoscopic transcanal facial nerve decompression in patients with traumatic facial nerve paralysis: from geniculate ganglion to mastoid segment.

OBJECTIVE: The current study evaluated the effectiveness of endoscopic transcanal facial nerve decompression in patients with post-traumatic facial nerve paralysis. METHODS: This retrospective study included 10 patients with post-traumatic complete facial nerve paralysis who underwent endoscopic transcanal facial nerve decompression. The surgical technique was explained step by step, and the surgical complications, hearing status and facial nerve function 12 months post-operatively were reported. RESULTS: Endoscopic transcanal facial nerve decompression allowed exposure of the geniculate ganglion to the mastoid segment. The facial nerve function improved from House-Brackmann grade VI to grades I and II in 8 of 10 (80 per cent) patients, and 2 patients experienced partial recovery (House-Brackmann grades III and IV). No severe complication was reported. CONCLUSION: Endoscopic transcanal facial nerve decompression, involving the nerve from the geniculate ganglion to the mastoid segment, is a safe and effective approach in patients with post-traumatic facial nerve paralysis.

Vestibular evaluation following cochlear implantation in patients with inner-ear anomalies.

OBJECTIVE: This cross-sectional study investigated vestibular function outcomes after cochlear implantation in patients with inner-ear anomalies. METHODS: Twenty-two patients with bilateral symmetric inner-ear anomalies and 28 patients with normal inner ears were included. All were congenitally or progressively deaf persons implanted unilaterally during the previous 15 years. Vestibular system function was assessed by vestibular-evoked myogenic potential and bithermal caloric tests. RESULTS: The vestibular-evoked myogenic potential abnormality rate in implanted ears with an inner-ear anomaly was 81.8 per cent, compared with 39.3 per cent in implanted ears with normal anatomy. In the non-implanted sides, the rate was 45.5 per cent (10 out of 22 cases) in the inner-ear anomaly patients compared with 17.9 per cent in patients with normal inner-ear structure. The respective abnormal caloric test rates in inner-ear anomaly versus normal anatomy patients were 81.8 per cent and 17.9 per cent (implanted ears), 77.3 per cent and 14.3 per cent (non-implanted sides). CONCLUSION: Inner-ear anomaly and implantation were both associated with more vestibular-evoked myogenic potential abnormalities; when occurring together, these factors showed a synergistic effect. Caloric test abnormality is mainly dependent on the presence of an inner-ear anomaly, but implantation is not associated with caloric abnormality.

Hearing status in patients with rheumatoid arthritis.

OBJECTIVE: Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors. METHODS: Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers. RESULTS: Pure tone audiometry results revealed a higher bone conduction threshold in the rheumatoid arthritis group, but there was no significant difference when evaluated according to the sensorineural hearing loss definition. Distortion product otoacoustic emissions related prevalence of conductive or mixed hearing loss, tympanometry values, acoustic reflexes, and speech discrimination scores were not significantly different between the two groups. Sensorineural hearing loss was significantly more prevalent in patients who used azathioprine, cyclosporine and etanercept. CONCLUSION: Higher bone conduction thresholds in some frequencies were detected in rheumatoid arthritis patients that were not clinically significant. Sensorineural hearing loss is significantly more prevalent in refractory rheumatoid arthritis patients.

Effect of noise pollution on testicular tissue and hormonal assessment in rat.

Many studies have focused on the effect of noise stress on the health. So far, few studies have been conducted on the effect of noise on reproductive system. The aim of study was to investigate the effect of noise pollution on morphometric parameters of testicular tissue and hormonal assessment (ACTH, cortisol and testosterone). In this study, 40 male rats were exposed to control, 95, 105 and 115 dB noise intensity for sixty days. At the end of study, blood sampling was performed and ACTH, cortisol and testosterone concentrations were assessed. The results showed that noise stress decreased testosterone levels in the 115 dB-treated group, while it increased the ACTH and cortisol levels. Histological sections of testis showed that the mean diameter of the seminiferous tubules and thickness of the germinal epithelium reduced compared to the control group. Also the ratio of the interstitial tissue area to the total testicular tissue area was increased significantly. Our study shows that noise stress may have negative influences on male fertility.

Prevalence of GJB2-associated deafness and outcomes of cochlear implantation in Iran.

OBJECTIVES: To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian children with cochlear implants, and to compare the outcomes of auditory perception and speech production in cochlear-implanted children with and without GJB2 mutation. MATERIALS AND METHODS: One hundred and sixty-six prelingually deaf children who had undergone cochlear implantation at the Iranian Cochlear Implant Center, Tehran, were selected from a pool of 428 implanted children. The prevalence of GJB2 gene mutations was assessed using nested polymerase chain reaction and direct sequencing. To enable comparisons, we also identified 36 implanted children with non-GJB2 deafness. Patients' speech perception and speech production were assessed using the Categorization of Auditory Performance and Speech Intelligibility Rating scales. RESULTS: Thirty-three of 166 probands (19.9 per cent) were found to have GJB2 deafness-causing allele variants and were diagnosed with DFNB1 deafness. Results also indicated a significant improvement in speech perception and production scores in both GJB2 and non-GJB2 patients over time. CONCLUSION: Children with GJB2-related deafness benefit from cochlear implantation to the same extent as those with non-GJB2-related deafness.

Viral infections detected by serology and PCR of perilymphatic fluid in children with idiopathic sensorineural hearing loss.

The etiology of sensorineural hearing loss (SNHL) in children may be viral. This cross-sectional study aimed to determine the role of viral infectious agents in children with idiopathic SNHL. Of 119 children with SNHL aged 3-168 months undergoing cochlear implant surgery at a hospital in Tehran, no cause could be established in 18 cases (15.1%). Cytomegalovirus (CMV) and herpes simplex virus (HSV) active infections (detected by DNA-PCR, confirmed by serology) were found in the perilymphatic fluid of 16.7% (3/18) cases of idiopathic SNHL. Serology was performed on blood samples from 11 of these cases: specific antibodies against CMV, Toxoplasma spp., HSV and rubella were determined in all cases; acute T. gondii infection was detected in 7 cases and rubella IgG was found in only 1 case. Neonatal screening for CMV, HSV and T gondii may be helpful in the Islamic Republi of Iran.

Viral infections detected by serology and PCR of perilymphatic fluid in children with idiopathic sensorineural hearing loss

The etiology of sensorineural hearing loss [SNHL] in children may be viral. This cross-sectional study aimed to determine the role of viral infectious agents in children with idiopathic SNHL Of 119 children with SNHL aged 3-168 months undergoing cochlear implant surgery at a hospital in Tehran, no cause could be established in 18 cases [15.1%]. Cytomegalovirus [CMV] and herpes simplex virus [HSV] active infections [detected by DNA-PCR, confirmed by serology] were found in the perilymphatic fluid of 16.7% [3/18] cases of idiopathic SNHL Serology was performed on blood samples from 11 of these cases: specific antibodies against CMV, Toxoplasmo spp., HSV and rubella were determined in all cases; acute T. gondii infection was detected in 7 cases and rubella IgG was found in only 1 case. Neonatal screening for CMV, HSV and T. gondii may be helpful in the Islamic Republic of Iran

Dynamic posturography for staging of patients with Ménière's disease.

OBJECTIVE: In recent years, techniques such as computerised dynamic posturography have facilitated objective evaluation of patients with balance disorders. In this study, we used computerised dynamic posturography for clinical staging of Ménière's disease. MATERIAL AND METHODS: We applied a computerised dynamic posturography sensory organisation test to 53 patients with American Academy of Otolaryngology criteria of Ménière's disease. The following parameters were considered in data analysis: computerised dynamic posturography scores for testing the patient's centre of gravity with a mobile surface, immobile visual surroundings and eyes closed, scores for testing with a mobile surface, mobile visual surroundings and eyes open, and vestibular ratio. We used four hearing threshold stages (although we had no patients at stage four). RESULTS AND DISCUSSION: This study did not observe a statistically significant correlation between audiometric hearing thresholds and computerised dynamic posturography scores. Computerised dynamic posturography scores showed statistically significant variation with time elapsed since the last vertigo attack. Although our results showed an increasing trend in computerised dynamic posturography scores as Ménière's disease activity level decreased from recent post-attack (i.e. less than one week since last attack) through late post-attack (i.e. one week to 60 days since last attack) to inactive (i.e. more than 60 days since last attack), these differences were not statistically significant.

Cochlear implantation in children with Jervell, Lange-Nielsen syndrome.

Jervell, Lange-Nielsen syndrome is a condition that causes profound hearing loss and disruption of the normal cardiac rhythm. This disorder is a form of long QT syndrome, a cardiac disorder that causes the cardiac muscle to take longer than usual to recharge between beats. A retrospective case study was performed to document cochlear implantation in three profoundly deaf children (two of them siblings) with Jervell, Lange-Nielsen syndrome. We discuss diagnosis and management of this syndrome and also the long-term performance of cochlear implantation in these Iranian patients, referring especially to the role of the ENT specialist in diagnosis and treatment. The collected data show that cochlear implantation can be relatively safely performed in patients with Jervell, Lange-Nielsen syndrome and that these children received significant benefit from cochlear implantation.

Cochlear implantation in prelingually deaf persons with additional disability.

OBJECTIVES: We aimed to identify the frequency with which the following conditions were present as a second disability in cochlear-implanted, prelingually deaf persons: mild and moderate mental retardation; learning disability; attention deficit/hyperactivity disorder; cerebral palsy; congenital blindness; and autism. We also aimed to document the development of auditory perception in patients having one of these additional disabilities. STUDY DESIGN: A retrospective study was designed to pursue the above aims. METHODS: We examined the records of 398 cochlear-implanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Patients were selected who showed a delay in motor, cognitive or emotional development. The selected cases were referred for psychological evaluation in order to identify patients with additional disabilities. We then compared these patients' auditory perception prior to and one year following cochlear implantation. RESULTS: A total of 60 (15 per cent) cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. These were classified as: mild mental retardation in eight cases (13.33 per cent); moderate mental retardation in five (8.33 per cent); learning disability in 20 (33.33 per cent); attention deficit/hyperactivity disorder in 15 (25 per cent); cerebral palsy in five (8.33); congenital blindness in three (5 per cent); and autism in four (6.66 per cent). All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients. CONCLUSION: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation. These patients require unique rehabilitation in order to achieve more auditory development.

Cochlear implantation in children with Waardenburg syndrome.

Waardenburg syndrome is an autosomal-dominant trait resulting from mutations occurring in different genes. It is often characterized by varying degrees of: congenital hearing loss; dystopia canthorum; synophrys; broad nasal root; depigmentation of hair (white forelock), skin or both; and heterochromic or hypochromic irides. A retrospective case study was done to assess speech perception, speech production, general intelligence and educational setting in six profoundly hearing-impaired children with Waardenburg syndrome (four with type I, one with type II and one with type III) ranging in age from two years to 14 years, seven months (mean = six years, six months). None of the patients had malformation of the cochlea and were implanted using Nucleus 22/24 and Med-el combi40+. Five out of the six cases were of average intelligence and one had a borderline intelligence quotient. The follow-up period ranged from one year, 10 months to six years, six months (mean = three years, six months) after implantation. The evaluation of auditory perception in patients was accomplished using the Persian Auditory Perception Test for the Hearing-Impaired, a Persian Spondee words test and the Categories of Auditory Performance Index. The Speech Intelligibility Rating test was used to evaluate speech production ability. All the patients' speech perception and speech intelligibility capabilities improved considerably after receiving the implants, and they were able to be placed in regular educational settings. Patients used their cochlear-implant devices whenever awake, implying that they benefitted from the devices. We suggest that any further expansion of cochlear-implantation criteria in children include those with Waardenburg syndrome.

Positional envelope as a response parameter in caloric testing.

Bithermal caloric testing was carried out in 57 normal subjects and 374 patients presenting with subjective complaints of vertigo over a 4-year period from December 1984 to December 1988. Responses were quantitatively assessed using a DEC PDP 11/73 laboratory minicomputer. Patients were classified as normal and abnormal according to caloric responses based on standard methods of calculating unilateral hypoexcitability and directional preponderance using the maximum slow component velocity. Results obtained from the slow component velocity for unilateral hypoexcitability and directional preponderance were compared to the same values obtained from the overall positional envelope calculated by an integration of the slow component velocity vs. time curve. Although duration of nystagmus varies extensively in normal subjects and has not proven clinically useful in identifying abnormalities, the integral of response amplitude over time gives a more complete description of the vestibular response. However, when compared to the maximum slow component velocity in abnormal patients, the positional envelope identified only 94 of the total 119 abnormalities (79%). Therefore, maximum slow component velocity is the more sensitive response parameter in identifying vestibular pathology.