Comparison of Carina active middle-ear implant with conventional hearing aids for mixed hearing loss.

OBJECTIVE: To compare the auditory outcomes of Carina middle-ear implants with those of conventional hearing aids in patients with moderate-to-severe mixed hearing loss. METHODS: The study comprised nine patients (six males, three females) who underwent middle-ear implantation with Carina fully implantable active middle-ear implants to treat bilateral moderate-to-severe mixed hearing loss. The patients initially used conventional hearing aids and subsequently received the Carina implants. The hearing thresholds with implants and hearing aids were compared. RESULTS: There were no significant differences between: the pre-operative and post-operative air and bone conduction thresholds (p > 0.05), the thresholds with hearing aids and Carina implants (p > 0.05), or the pre-operative (mean, 72.8 ± 19 per cent) and post-operative (mean, 69.9 ± 24 per cent) speech discrimination scores (p > 0.05). One of the patients suffered total sensorineural hearing loss three months following implantation despite an initial 38 dB functional gain. All except one patient showed clinical improvements after implantation according to quality of life questionnaire (Glasgow Benefit Inventory) scores. CONCLUSION: Acceptance of Carina implants is better than with conventional hearing aids in patients with mixed hearing loss, although both yield similar hearing amplification. Cosmetic reasons appear to be critical for patient acceptance.

Transmastoid labyrinthectomy for disabling vertigo after cochlear implantation.

OBJECTIVE: To document the use of transmastoid labyrinthectomy in the treatment of disabling vertigo after unilateral cochlear implantation. METHODS: A 58-year-old man with severe-to-profound bilateral sensorineural hearing loss secondary to chronic otitis media underwent cochlear implantation in his right ear with a Pulsar Med-El device. The surgery was uneventful and the electrode was positioned correctly. He had episodic vertigo three months after implant surgery, and medical treatment and aggressive vestibular rehabilitation did not relieve the vertigo attacks. RESULTS: Right transmastoid labyrinthectomy was performed one year after cochlear implantation. The patient's symptoms were immediately relieved, and cochlear implant function was not adversely affected at follow up after three years. CONCLUSION: Transmastoid labyrinthectomy seems to be an effective, safe method for ablating the vestibular end organ after unilateral cochlear implantation.

Facial reanimation with end-to-end hypoglossofacial anastomosis: 20 years' experience.

OBJECTIVE: This study aimed to evaluate retrospectively the results of experience with end-to-end anastomosis of cranial nerves VII and XII, performed due to transection of the facial nerve during acoustic neuroma removal. METHODS: We assessed the facial reanimation results of 33 patients whose facial nerves had been transected during acoustic neuroma excision via a retrosigmoid approach, between 1985 and 2006, and who underwent end-to-end hypoglossofacial anastomosis. We compared the facial nerve functions of patients receiving short term (two to three years) and long term (more than three years) follow up, and we assessed any complications of the anastomosis. RESULTS: A House-Brackmann grade III facial function was achieved in 46.2 and 86.4 per cent of the patients in the short and long term, respectively. House-Brackmann grade IV facial function was achieved in 53.8 and 13.6 per cent of the patients in the short and long term, respectively. There was a statistically significant difference between the facial recovery results, comparing the short and long term follow-up periods (p = 0.03). Disarticulation was the most common complication, seen in 19 (57.6 per cent) patients; numbness of the tongue was the next commonest (10 (30.3 per cent) patients). None of the patients developed dysphagia. CONCLUSION: Despite such morbidities as disarticulation and tongue numbness, end-to-end hypoglossofacial anastomosis is still an effective procedure for the surgical rehabilitation of static and dynamic facial nerve functions. Significant improvement in facial nerve function can occur more than three years post-operatively.

Congenital neck masses in children and their embryologic and clinical features.

Congenital neck masses in children and their embryologic and clinical features. Neck masses of congenital origin can be diagnostic and therapeutic challenges for internists, paediatricians and surgeons. Treatment modalities of congenital neck masses are different depending on their nature, symptoms and location. Differential diagnosis includes a variety of diseases that can cause cervical masses such as infectious and neoplastic neck tumours. Our objective is to review the embryologic and clinical features of some of the most common congenital neck masses such as the haemangioma, branchial cleft anomalies, thyroglossal duct cyst, ectopic thyroid, congenital midline cervical cleft, congenital cervical teratoma, lymphangioma, cervical thymic cyst, dermoid cyst and congenital muscular torticollis.

Absence of contralateral suppression of transiently evoked otoacoustic emissions in fibromyalgia syndrome.

OBJECTIVE: To assess contralateral suppression of transiently evoked otoacoustic emissions in patients with fibromyalgia syndrome and normal hearing. METHODS: Twenty-four female patients with fibromyalgia syndrome and 24 healthy female controls with normal hearing were assessed using pure tone audiometry and transiently evoked otoacoustic emissions. RESULTS: All patients with fibromyalgia syndrome and all controls had normal hearing on pure tone audiometry. In the patients with fibromyalgia syndrome, the mean transiently evoked otoacoustic emission amplitude was 15.5 +/- 4.8 dB. The mean transiently evoked otoacoustic emission amplitudes after contralateral suppression was 15.5 +/- 4.9 dB. There was no statistically significant difference between the transiently evoked otoacoustic emission amplitudes measured before and after contralateral suppression (p > 0.05). In the controls, the mean transiently evoked otoacoustic emission amplitude was 12 +/- 5 dB. The mean transiently evoked otoacoustic emission amplitudes after contralateral suppression was 11 +/- 4.7 dB. There was a statistically significant decrease in transiently evoked otoacoustic emission amplitudes after contralateral suppression (p < 0.01). CONCLUSION: The mechanisms related to contralateral suppression of transiently evoked otoacoustic emissions seem dysfunctional in fibromyalgia syndrome. This dysfunction may be at the brain stem level, where the medial superior olivary complex is located, or at the synapses of medial superior olivary complex fibres with the outer hair cells in the cochlea. Demonstration of lack of contralateral suppression of transiently evoked otoacoustic emissions can be used as a diagnostic tool in patients with fibromyalgia syndrome.

Subclinical dysfunction of cochlea and cochlear efferents in migraine: an otoacoustic emission study.

Otoacoustic emission (OAE) testing enables us to identify the cochlear component of a hearing disorder and to monitor objectively minute changes in cochlear status undetectable by other audiological methods. Contralateral sound-induced suppression is mediated by medial superior olivary complex efferents which induce hyperpolarization counteracting the amplifying effects of outer hair cell (OHC) activity. The aim of this study was to assess functions of cochlea and its efferents in migraine using OAE testing and contralateral suppression of transiently evoked OAEs (TEOAE). Fifty-three migraineurs (106 ears) and 41 healthy subjects (82 ears) were included and pure tone audiometry (PTA), speech discrimination scores (SDS), distortion product OAE (DPOAE), TEOAE and contralateral suppression of TEOAEs were tested. PTA and SDS of migraineurs and controls were not different (P > 0.05). DPOAEs were tested between 1 and 6 kHz and a significant difference was detected only at 5 kHz frequency, where DPOAE amplitudes in migraine with aura (MA) were lower than in controls (P < 0.03). The mean amplitudes of TEOAEs were statistically insignificant between controls and migraine groups. Contralateral sound stimulus induced significant decrease in amplitudes of TEOAE (P = 0.005) in controls. In patients with migraine without aura and MA, mean amplitudes of TEOAEs were not suppressed by contralateral sound stimulus (P > 0.05). As PTA, SDS and DPOAE tests demonstrate normal functioning of inner ear between 1 and 4 kHz, absence of suppression of the TEOAEs by contralateral sound stimulation indicates the presence of dysfunction either in the medial olivocochlear complex in the brainstem or at the synaptic transmission between olivocochlear efferents and OHCs in the cochlea. Disruption in the contralateral suppression may be one of the mechanisms predisposing to the phonophobia symptom associated with migraine headache.

Impact of prognostic factors on recovery from sudden hearing loss.

OBJECTIVE: To define the impact of patient-related and audiovestibular parameters on the prognosis of sudden hearing loss. METHODS: Eighty-three patients were included in this retrospective study. All were treated medically. We recorded the patients' demographic parameters, systemic diseases, time elapsed between onset of sudden hearing loss and initiation of treatment, tinnitus, vestibular symptoms, type of initial audiogram, pure tone averages and speech discrimination scores. For all patients, audiological measurements were performed on initial admission and at the completion of treatment on the 10th day. RESULTS: There was no correlation between the hearing gain and recovery rate scores and patients' gender or age (p>0.05). However, a correlation was found between gender and relative hearing gain. Vertigo was not correlated with hearing gain and recovery rate scores (p0.05); time elapsed between onset of sudden hearing loss and initiation of treatment (p>0.05); type of audiogram on initial admission (p>0.05), except for midfrequency type of audiogram; and tinnitus (p>0.05). CONCLUSIONS: The outcome of sudden hearing loss was unaffected by systemic disease, tinnitus or type of audiogram (except for midfrequency type). The following were poor prognostic factors in the outcome of sudden hearing loss: female gender, presence of vertigo, initiation of treatment more than seven days after onset of hearing loss, and >40 dB hearing loss on admission.

Influence of the relapse and remission periods on hearing status in children with minimal change nephrotic syndrome.

OBJECTIVE: Minimal change nephrotic syndrome (MCNS) is characterized by the onset of NS (Nephrotic Syndrome) without systemic disease, hypocomplementemia, or other serious signs of renal disease. Hearing status is not very well known in MCNS. Our objective was to address this question and to find out remission and relapse periods of the syndrome would affect the hearing of the patients. METHODS AND PATIENTS: Otologic status of 26 children with clinical MCNS was investigated in relapse and remission periods using audiometry and tympanometry. The pure tones that were obtained at the frequencies 250, 500, 1000, 2000, 4000 and 6000 Hz were noted. Pure tone averages (PTAs) were calculated at 500, 1000, 2000 and 4000 Hz frequencies. RESULTS: In both remission and relapse periods, PTA of the patients did not change and was 13 dB. The frequency specific pure tone results were not significantly different between the right and left ears of the patients as well as between the remission and relapse periods (p > 0.05). In the relapsing and remission periods, type A tympanogram was encountered in 86.4% and 92.3% of the ears, respectively. Type B tympanogram was encountered in 11.5% and 3.8% of the ears in the relapsing and remission periods, respectively. Type C tympanogram was encountered in 3.8% of the ears both in the relapsing and remission periods. Differences between the tympanometry results were not significant (p > 0.05). CONCLUSION: MCNS in childhood is not associated with an alteration in the hearing status, both in remission and relapse periods of the disease.

Relation between actinomycosis and histopathological and clinical features of the palatine tonsils: a comparative study between adult and pediatric patients.

OBJECTIVE: To find out the incidence of actinomyces in the palatine tonsil of the adult and pediatric patients, and evaluate our results relevant to the clinical diagnoses and histopathological features of tonsillectomy specimens. METHODS: The medical records of 1820 tonsillectomies, which were performed in the Department of Otolaryngology between 1987 and 2002 were retrospectively reviewed. The paraffin embedded specimens of the patients were also obtained for histopathologic assessment, and Hematoxylin-Eosin stained sections re-evaluated mainly for cryptitis and actinomycosis. RESULTS: There was actinomyces in 122 (6.7%) of 1820 patients, of whom 62 were adult and 60 were pediatric patients. That is, actinomyces was present in 62 (10.9%) of 568 adult patients, and 60 (4.8%) of 1252 pediatric patients. The rate of actinomyces was significantly higher in adults than children (p < 0.01). There was no correlation between the clinical diagnoses and the histopathologic parameters assessed (p > 0.05). On histopathology, the cryptitis was encountered in 53 (85%) of 62 adult patients and 47 (78%) of 60 pediatric patients with actinomyces (p > 0.05). The rate of cryptitis was significantly higher in the presence of actinomyces both in adult and pediatric patients (p < 0.05). CONCLUSION: There was no correlation between the clinical diagnosis of tonsillar disease and the presence of actinomycosis both in adult and pediatric patients. Histopathologic findings of adult and pediatric tonsillectomy material was similar except for the higher rate of actinomycosis in the adults than in children. However, cryptitis was a substantial finding in the presence of actinomycosis. Based on the significant association of cryptitis with actinomycosis, cyptitis can be considered a histopathologic indicator for tonsillar actinomycosis.

Assessment of the ear and otoacoustic emission findings in fibromyalgia syndrome.

OBJECTIVE: We aimed to assess otoacoustic emission (OAE) findings in fibromyalgia (FM) syndrome. METHODS: Thirty-two ears of 16 female patients with FM syndrome and 30 ears of 15 healthy female controls were also included in the study. Pure tone audiometry, speech discrimination testing, tympanometry and otoacoustic emission testing (both transiently evoked and distortion product) were performed. RESULTS: There was no significant difference between the pure tone hearing results of the patients and controls (p > 0.05). There was no significant difference between the distortion-product-otoacoustic emission results of the patients and controls. Audiologic findings of the patients with and without otologic symptoms were not significantly different than controls (p > 0.05). CONCLUSION: Although FM patients generally have subjective symptoms related to ear, clinical or laboratory assessments usually fail to find out any objective finding related to these subjective symptoms. The otologic functions seem spared in FM syndrome.

Neurotological status in Behçet's disease and its ophthalmological correlates.

OBJECTIVE: To assess neurotological status and its ophthalmological correlates in Behçet's disease. STUDY DESIGN: A cross sectional study in which 17 patients with Behçet's disease and 13 healthy controls were included. PATIENTS AND METHODS: The patients were divided into two groups: those with and without end stage ocular involvement. Neurotological status was evaluated with audiological tests (pure tone and speech audiometry, tympanometry, short increment sensitivity index, tone decay, and evoked response audiometry), and Dix-Halpike positional testing. RESULTS: The positional test was normal. The audiological test results of the patients with and without end stage ocular involvement were not significantly different (p>0.05). A sloping audiogram with bilateral symmetric and mild sensorineural hearing loss was the main audiogram obtained. There was no relationship between the presence or absence of the end stage ocular involvement and the otological parameters studied excluding I-III interval on evoked response audiometry (p>0.05). CONCLUSION: Although the main underlying pathogenetic factor in Behçet's disease is the autoimmune vasculitis, the mechanisms involved in the pathogenesis of neurological and ocular damage may be different. Alternatively, the differential involvement of certain organ systems may not reflect the nature of the disease process itself, but rather the manner in which each organ responds to injury.

T102C polymorphism of the 5-HT2A receptor gene may be associated with temporomandibular dysfunction.

OBJECTIVE: To assess whether a relationship existed between the T102C polymorphism of 5-HT2A receptor gene and temporomandibular dysfunction. METHODS: Sixty-three patients with temporomandibular dysfunction, and 54 healthy volunteer controls were included in the study. Molecular analysis of the T102C polymorphism of the 5-HT2A receptor gene was performed using PCR technique. RESULTS: The C/C genotype was over represented in the patients whereas T/T genotype was over represented in the controls (P < 0.05). The genotype distribution of the patients who had temporomandibular dysfunction was not different than those who did not have temporomandibular dysfunction (P > 0.05). CONCLUSION: The T102C polymorphism may be involved in the etiology of temporomandibular dysfunction. The overrepresentation of the C/C variant of 5-HT2A receptor gene in temporomandibular dysfunction suggests a possible role of the serotonergic system in this disease, particularly at the receptor level.

Assessment of mastoid air cell size versus sigmoid sinus variables with a tomography-assisted digital image processing program and morphometry.

We assessed the mastoid air cell size and variables of the sigmoid sinus in healthy ears and ears with chronic otitis media (COM). Thirty-eight patients with unilateral COM [15 with cholesteatoma (COM/+) and 23 without cholesteatoma (COM/-)], and 20 subjects with healthy ears, were included in the study. Assessment was performed using a quantitative digital image processing computed tomography (CT) program, and the volume of the mastoid bone was measured using the morphometric method of Cavalieri. In both COM/+ and COM/- patients the sigmoid to suprameatal spine distance and mastoid size were greater on the healthy side than on the diseased side ( p<0.05). The distance and area were significantly greater in the healthy control subjects than in either the healthy or the diseased ears of the patients with COM ( p<0.05). In the healthy ears of COM patients, there was significant correlation between the sigmoid to suprameatal spine distance and air cell size and mastoid volume ( p<0.05). In the diseased ears of COM patients, this correlation was absent ( p>0.05). The sigmoid sinus shape was of the half-moon type (62%), protrusive type (22%) and saucer type (16%). The digital image processing CT program allowed us to estimate the individual area of the air and soft tissue filled mastoid air cells. The mastoid size in both intact and disease ears of COM patients was smaller than in the healthy controls. The mastoid size may be determined genetically. However, environmental factors such as infection may also affect the mastoid size. Therefore, both genetic and environmental factors may be related to COM as far as the size of the mastoid air cells is concerned.

Körner's septum (petrosquamosal lamina) and chronic ear disease.

To assess the clinical significance of Körner's septum (KS) or petrosquamosal (PS) lamina in different chronic ear pathologies, a surgical and anatomic study was carried out, in which the significance of KS was assessed in various otologic diseases. Of 639 patients who underwent ear surgery between 1998 and 2001, 426 were included in the study. The diagnoses were chronic otitis media without cholesteatoma (COM), tympanosclerosis (TS), adhesive otitis media (AOM) and cholesteatoma (COL) in 160, 100, 70 and 96 patients respectively. The operative records of the patients were reviewed for the presence of KS. There was antral blockage by KS in all patients who also had Cog (the middle portion of the PS lamina). KS was mostly seen in cases of TS. The prevalence of KS was not significantly different when the result of the COM group was compared with that of the COL and AOM groups ( p>0.05). The prevalence of KS was significantly higher in patients with TS than in those with COM. Both antral and epitympanic blockages were more prevalent in cases of TS than in those of COM (chi(2) =3.9, p =0.04 and chi(2) =6.6, p =0.01 respectively). There is a significant association between the presence of KS and TS. Based on this finding, it is recommended that a mastoidectomy be performed in cases of severe tympanosclerosis in order to remove KS. How the removal of this anatomic impact will affect the natural course of the disease is still a question that needs further investigation.

Important anatomic variations of the sinonasal anatomy in light of endoscopic surgery: a pictorial review.

Advances in surgical techniques and instrumentations, utilization of the imaging tools, and understanding the regional anatomy have served to make functional operations with less complications in the paranasal sinus area. In the context of this article, some of these anatomical structures and their variations were reviewed through images. These structures were as follows: course of the anterior ethmoidal artery; roof of the ethmoid; lamina papyracea; uncinate process; optic nerve; and internal carotid artery.

Significance of the catechol-O-methyltransferase gene polymorphism in migraine.

The objective was to assess the significance of the catechol-o-methyltransferase (COMT) enzyme polymorphism in migraine. For this reason, 62 migraineurs and 64 healthy volunteers were included in the study. The analysis of COMT polymorphism was performed using PCR. The H/H genotype was more frequent in the control group than in the patients group (P=0.032). The homozygous or heterozygous L allele was over represented in the migraineurs compared with the controls (P=0.013). The L/L genotype was over represented in the migraineurs who also had a family history of migraine (P=0.003). There was no relationship between aura and COMT genotypes. In conclusion, the COMT polymorphism may be of potential pharmacological importance regarding the individual differences in the metabolism of catechol drugs in migraineurs. Although altered catecholamine activity due to polymorphism of COMT gene may be one of the mechanisms involved in the pathogenesis of migraine, these mechanisms are not related to presence or absence of aura.

The Chiari III malformation: an unusual and asymptomatic variant in an 11-year old child.

Chiari III malformation is an extremely rare condition, and is characterized by the hindbrain herniation into a low occipital or high cervical encephalocele together with the pathologic and imaging features of the Chiari II malformation. In this report, an unusual variant of the Chiari III malformation was diagnosed in an 11-year-old girl. She had an encephalocele operation when she was a newborn, and has been asymptomatic since then. The clinical and imaging findings of this case were presented.

Association of the T102C polymorphism of 5-HT2A receptor gene with aura in migraine.

OBJECTIVE: To find out the significance of the 5-HT2A receptor gene polymorphism in migraine. STUDY DESIGN: A PCR study in which 61 migraineurs and 44 healthy controls were included. METHODS: The T102C polymorphism of the 5-HT2A receptor gene was studied. The results of the migraineurs and controls were compared. The relationship between the gene polymorphism and aura was also assessed. RESULTS: The representations of the 5-HT2A genotypes were similar in migraineurs and controls (p>0.05) as well as in the male and female migraineurs (p>0.05). The family history of migraine did not associate with 5-HT2A receptor gene polymorphism (p>0.05). There was a significant relationship between the presence of C/C genotype and migraine with aura (p=0.02) while C/T and T/T genotypes were over represented in the patients with migraine without aura (p<0.01). CONCLUSION: The T102C polymorphism of the 5-HT2A receptor gene is not directly related to the increased risk of migraine. The associations between the genotypes of this gene and aura may suggest that 5-HT2A receptor gene polymorphism may be involved in determining the subtypes of or accompanying symptoms in the migraine disease.

Significance of the auditory tube angle and mastoid size in chronic ear disease.

The aim of this study was to determine whether a relationship exists between mastoid size and auditory tube angle (ATA) and chronic ear disease. A radiological and clinical study was undertaken of the temporal bones of 36 subjects. There were 24 patients with unilateral chronic otitis media (COM) and 12 healthy controls (HC): the contralateral healthy ears of the COM patients were also used as control (CLHC). From each CT scan the distance between Henle spine and the sigmoid sinus (H-SS) and the ATA was assessed. The angle between a longitudinal line bisecting the transverse length of the external auditory canal and the longitudinal axis of the AT was calculated. A direct correlation between H-SS and ATA, both in COM and HC (p < 0.01), was observed, but not in CLHC (p > 0.05). There was no significant difference in the ATA results between COM and CLHC, or between COM and HC (p > 0.05), however there was a significant difference between CLHC and HC (p = 0.02). H-SS distances were not significantly different between COM and CLHC, or COM and HC, or CLHC and HC (p > 0.05). An association between ATA and mastoid size in both healthy and diseased ears was observed. The anatomical relationships of the AT, mastoid and middle ear, which form a functional unit, are not significantly important in chronic ear disease.

Cycline-dependent kinase inhibitor, p27 (KIP1), is associated with cholesteatoma.

OBJECTIVE: Cyclin-dependent kinases (CDKis) can arrest the cell cycle, which in turn inhibits the cell proliferation. P27 (KIP1) is a CDKi and acts as a tumor suppressor gene. In this study, we aimed to investigate the role of p27 CDKi in cholesteatoma, a disease characterized by the presence of hyperproliferative squamous epithelium. STUDY DESIGN: Immunohistochemical staining of 15 cholesteatoma and 18 control ear canal skin samples, which were taken intraoperatively, was performed for p27 positivity. METHODS: The monoclonal antibodies to p27 were used for immunohistochemical staining of the sections. The streptavidin-biotin horseradish method was used. The number of cells staining positive for p27 was calculated, and the intensity of p27 positivity was graded. RESULTS: P27 positivity was obtained in 9 (50%) of 18 skin tissues. In the cholesteatoma tissues, p27 positivity was found only in 2 (13.3%) tissue samples. The difference between the groups were statistically significant (P =.03). The mean numbers of p27 positivity were 11.8 +/- 15.5 and 1.4 +/- 3.8 (mean +/- standard deviation) in the skin and cholesteatoma samples, respectively. This difference was also statistically significant (P =.02). The p27 results of primary and secondary cholesteatoma samples were not significantly different (P =.3). The results of p27 were not related to the gender of the patients (P =.8). CONCLUSION: P27 is involved directly or indirectly in the occurrence of cholesteatoma. Alterations of p27 levels in keratinocytes can influence the proliferative state of the keratinocytes. Altered p27 levels in cholesteatoma may suggest a molecular pathology in cholesteatoma. The search for significance of CDKis seems promising to better understand the pathogenesis of cholesteatoma.