Hearing impairment and ear anomalies in craniofacial microsomia: a systematic review.

The aim of this systematic review was to review the literature on hearing impairment and ear anomalies in patients with craniofacial microsomia and to determine their prevalence. Sixty-two records including 5122 patients were included. Ear anomalies were present in 52-100% of patients. The most reported external ear malformations were microtia, pre-auricular tags, and atresia of the external auditory canal. Ossicular anomalies were the most reported middle ear malformations, whereas the most reported inner ear malformations included oval window anomalies, cochlear anomalies, and anomalies of the semicircular canals. Hearing loss in general was reported in 29-100% of patients, which comprised conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. Between 21% and 51% of patients used hearing aids, and 58% underwent a surgical intervention to improve hearing. The relationship between different phenotypes of craniofacial microsomia and the type and severity of hearing loss is mostly unclear. In conclusion, the high prevalence of ear and hearing anomalies in patients with craniofacial microsomia underlines the importance of audiological screening in order to facilitate individual treatment.

Multicenter Study of Congenital Middle Ear Anomalies. Report on 246 Ears.

OBJECTIVES/HYPOTHESIS: Congenital middle ear anomalies represent a relatively rare condition. This study aimed to describe the characteristics and the surgical outcomes for patients with middle ear anomalies. METHODS: A multicenter study was conducted of consecutive patients with congenital middle ear anomalies who underwent primary surgical treatment between January 2008 and December 2017. Demographics, surgical procedures, and audiometric data were registered into the institutional database. Hearing changes and postoperative air-bone gap (ABG) were evaluated 1 year after surgery. RESULTS: A total of 246 patients (246 ears) (median age: 14 years, range: 4-75 years old) were included in this study. Anomalies were subdivided using the Teunissen and Cremers classification: 53 ears (22%) were categorized as class I, comprising only stapes ankylosis; 35 ears (14%) as class II, having ossicular chain anomalies with stapes ankylosis; 139 ears (57%) as class III, having ossicular chain anomalies with a mobile stapes-footplate; and 19 ears (8%) as class IV, with aplasia of the oval window. Evaluation of hearing outcomes for 198 ears with more than 1 year of follow-up revealed that good postoperative ABG (≤20 dB) was achieved in 82% of class I, 68% of class II, 74% of class III, and 23% of class IV anomalies. The postoperative ABG in class IV was significantly worse than in class I (P < .001) or class III (P < .01). CONCLUSIONS: This study demonstrated that class III anomalies comprised the majority of middle ear anomalies and surgical outcomes for class IV anomalies are unfavorable. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2323-E2328, 2021.

Reconstruction of the external auditory canal using full-thickness rolled-up skin graft after lateral temporal bone resection for T1 and T2 external auditory canal cancer.

OBJECTIVE: To present our results of the external auditory canal (EAC) reconstruction procedure using rolled-up full-thickness skin graft with tympanoplasty after lateral temporal bone resection (LTBR) for early-stage EAC carcinoma. PATIENTS AND METHODS: A retrospective review of 15 patients who had undergone LTBR with reconstruction of the EAC for T1 and T2 EAC cancer between 2016 and 2020. RESULTS: Postoperative mean air-bone gap was 30.7 decibel hearing level. Although a few patients experienced chronic granulation, persistent otorrhea, and/or laterization of the tympanic membrane, most patients showed no serious complications related to the EAC reconstruction. CONCLUSION: EAC reconstruction using a full-thickness skin graft in combination with tympanoplasty is useful for minimizing the hearing loss, maintaining the cosmetic appearance, and facilitating the observation into the ear cavity.

Renal Syndromic Hearing Loss Is Common in Childhood-onset Chronic Kidney Disease.

BACKGROUND: Hearing loss (HL) in children may adversely affect their development. HL is more prevalent in patients with chronic kidney disease (CKD) than in the general population. This study evaluated the prevalence of HL and its underlying diseases in patients with childhood-onset in CKD. METHODS: In this retrospective study of a tertiary referral center, childhood-onset CKD patients (stage 2-5, age at onset of renal symptom < 18 years) were recruited. We referred to the "renal" syndromic HL as cases with genetic or syndromic diseases, or extra-renal anomalies in addition to HL and CKD. RESULTS: A total of 421 patients (male:female = 279:142) were reviewed according to the causes of CKD: congenital anomalies of the kidney and urinary tract (CAKUT; n = 184, 43.7%), glomerulopathies (GP; n = 105, 24.9%), cystic kidney diseases (CYST; n = 39, 9.3%), perinatal problems (PP; n = 29, 6.9%), and others (n = 64, 15.2%). HL was detected in 82 (19.5%) patients, including 51 (12.1%) patients with sensorineural hearing loss (SNHL), 30 (7.1%) with conductive hearing loss (CHL), and 1 patient with mixed HL. The prevalence of HL in each group was as follows: 16.8% in the CAKUT group, 28.6% in the GP group, 12.8% in the CYST group, 24.1% in the PP group, and 14.1% in the others group. HL was more common in higher CKD stages, especially CHL in end-stage renal disease. SNHL was more prevalent in CKD from GP. Of the 82 patients with HL, 50% had renal syndromic HL: 58.8% of SNHL and one-third of CHL were renal syndromic HL. CONCLUSION: One-fifth of the childhood-onset CKD had HL. Collectively, renal syndromic HL comprised half of the HL in this study. To improve the quality of life in patients with childhood-onset CKD, we suggest that HL should be considered, requiring surveillance, and if necessary, early intervention.

Association of rheumatoid arthritis with hearing loss: a systematic review and meta-analysis.

Rheumatoid arthritis (RA) is a systemic autoimmune disease affecting up to 1% of the worldwide population. RA is associated with multiple extra-articular manifestations (EAMs). Middle ear, cochlea and the auditory nerve are suspected sites of RA activity and hearing loss is a possible novel EAM of RA. Objective was to investigate the association of RA with the different subtypes of hearing loss. This systematic review was performed according to the PRISMA guidelines. A random effects model meta-analysis was conducted and the I2 was used to assess heterogeneity. Twelve studies comprising 20,022 RA patients and 79,244 controls were included in this systematic review. All studies were observational and were rated to a moderate rate of bias. RA patients had nearly fourfold increased odds of sensorineural hearing loss (SNHL) compared with controls (OR 3.42; 95% CI 2.50-4.69; I2 = 13). RA patients also had a significantly increased risk of SNHL (RR 2.28; 95% CI 1.88-2.76; I2 = 0). RA patients did not have increased odds of conductive hearing loss (CHL) and mixed hearing loss (MHL) (OR 1.36; 95% CI 0.52-3.55; I2 = 22); (OR 2.73; 95% CI 0.78-9.58; I2 = 0%). RA is significantly associated with SNHL. RA is not associated with CHL and MHL. Early screening of RA patients with pure tone audiometry should be considered.

Dosimetric parameters associated with conductive or sensorineural hearing loss 5 years after intensity-modulated radiation therapy in nasopharyngeal carcinoma.

BACKGROUND: Most previous studies are separate dosimetric analyses of conductive or sensorineural hearing loss, and they are not conducive to a comprehensive assessment of auditory radiation damage. AIMS/OBJECTIVES: Our study aimed to evaluate the long-term incidence of sensorineural hearing loss (SNHL) or conductive hearing loss (CHL) in patients with nasopharyngeal carcinoma (NPC) after intensity-modulated radiation therapy (IMRT), and to investigate the relationship between SNHL or CHL and patient factors, treatment-related factors, and radiation dose parameters. MATERIAL AND METHODS: Seventy patients (117 ears) with NPC, who were also treated with IMRT in our hospital from 2006 to 2014, were retrospectively analyzed. Radiation doses to the Eustachian tube (ET), middle ear (ME), cochlear (Co), and internal auditory canal (IAC) were assessed. Pure tone audiometry and impedance audiometry were performed before and during the follow-up period. The relationships between low-frequencies (0.5-2 kHz) or high-frequency (4 kHz) SNHL/CHL and radiotherapy dose parameters were analyzed. RESULTS: Of the 117 ears studied, 7.69% had low-frequency SNHL, 35.9% had high-frequency SNHL, 23.93% had low-frequency CHL, and 18.80% had high-frequency CHL. The incidence of high-frequency CHL was higher in the T4 group than in the T (1-3) group (p < .05). When IAC Dmax > 42.13 Gy or IAC Dmean > 32.71 Gy, the risk of high-frequency SNHL increased in NPC patients. When ME Dmax > 44.27 Gy, ME Dmean > 29.28 Gy, or ET Dmax > 57.23 Gy, the risk of high-frequency CHL in NPC patients increased. CONCLUSIONS AND SIGNIFICANCE: SNHL and CHL remain common ear complications after IMRT for NPC. IAC Dmax, IAC Dmean, ME Dmax, ME Dmean, and ET Dmax all need to be carefully considered during the IMRT treatment protocol.

Malformations of the lateral semicircular canal correlated with data from the audiogram.

OBJECTIVES: Lateral semicircular canal (LSCC) malformations  are one of the most common inner ear malformations. The purpose of this study is to analyze the prevalence and type of hearing losses associated with LSCC malformations, compared to a control group. MATERIALS AND METHODS: We retrospectively included 109 patients (166 ears) presenting with a CT-confirmed LSCC malformation, compared to a control group (24 patients). The bony island surface and the width of the inner portion of the LSCC were measured to confirm the malformation. There results were correlated to audiogram data: sensorineural (SHNL), mixed (MHL) or conductive hearing loss (CHL) by an otologist. RESULTS: In the LSCC group, 60.9% of patients presented with an audiogram-confirmed hearing loss, especially SNHL (39.2%, n = 65) and MHL (12.7%, n = 21). Hearing was normal in 39.2% (n = 65) of the cases. Bilateral LSCC malformations (n = 57) were frequently associated with hearing loss (80.7%), SNHL in most of the cases (33.3%). Unilateral LSCC malformations were associated with hearing alterations (51.9%, n = 27), but we also observed a high rate (81%, n = 42) of contralateral abnormalities of the audiogram. CONCLUSION: LSCC malformations are commonly associated with hearing loss (61%), especially SHNL (39%). The high rate (81%) of contralateral hearing disturbances in unilateral LSCC malformations should be taken into account in the patient's daily life to avoid triggering or exacerbating any hearing loss. Otologists and radiologists must cooperate to ensure that all malformations are correctly described on CT, especially to improve the patient's education regarding hearing preservation.

Hearing status and behavioural patterns among school aged children with cleft lip and/or palate.

OBJECTIVE: There is a dearth of studies on long term hearing status and behavioural patterns among cleft lip and/or palate children after their primary lip and palate closure in Malaysia. This study describes the audiology status and behavioural patterns in a group of school aged children with cleft lip and/or palate. METHOD: A cross sectional study was carried out where caretakers of cleft lip and/or palate were asked to complete the translated Malay language version of Strength Difficulties Questionnaire. The hearing status of the children was analyzed based on recent pure tone audiometric and tympanogram results. The patients' age, gender, type of cleft pathology, age of palatal surgery and behavioural patterns were examined for their potential relationship with hearing status. RESULTS: A total of 74 children (148 ears) aged between 7 and 17 years with cleft lip and/or palate were recruited. The result showed 37 ears (25.0%) had hearing loss with majority suffered from mild conductive hearing loss. There were 16 ears (10.8%) that had persistent middle ear effusion. Hearing improvement occurred when palatal repair was performed at the age of less than 1 year old. (p = 0.015) There was no significant relationship between patients' gender, age, type of cleft and history of myringotomy with their hearing status. In terms of behavioural patterns, 16.3% were abnormal for total behavioural score, 39.2% for peer problem and 17.6% for conduct problem. For prosocial behaviour, 16.3% were rated low and very low. There was fair correlation between age and hyperactivity problems (r = 0.44). Patients' gender, type of cleft pathology, had been teased apart and hearing status was found not related to behavioural problems. CONCLUSION: Cleft lip and/or palate patients have a good longterm hearing outcome. Majority had normal hearing and if there is hearing impairment, it is only a mild loss. Early palatal repair surgery before the age of 1 year can significantly reduce the risk of hearing loss. Cleft lip and/or palate patients experienced peer problems. There was no significant correlation between behavioural difficulty and hearing status among school-aged children with cleft lip and palate.

Cilia and Ear.

OBJECTIVE: To investigate the prevalence of otological complications derived from primary ciliary dyskinesia (PCD) in adulthood. METHODS: Twenty-three patients with diagnosed PCD underwent medical history aimed at recording the presence of ear, nose, and throat manifestations (ENT) and any surgical treatments. The ENT objectivity was annotated, and then patients were subjected to audiometric test, tympanometry, registration of otoacoustic emission, and vestibular evaluation. RESULTS: Otitis media with chronic middle ear effusion (OME) during childhood was reported in 52% of the subjects, no patient had undergone ear surgery, and only 2 patients had an episode of otitis in the last year. Eleven of 23 patients showed normal hearing, 11 had a conductive hearing impairment, and 1 showed a severe sensorineural hearing loss unrelated to the syndrome. The bilateral stapedial reflex was only found in all cases of normoacusia and type A tympanogram, distortion product otoacoustic emissions (DPOAE) were present in 8 patients, and no patient had vestibular alterations. CONCLUSION: Our study confirms a very frequent prevalence of OME in PCD during childhood. Careful monitoring of otological complications of the syndrome is always desirable, also given the high presence in adults of other manifestations in the upper airways, such as chronic rhinosinusitis and nasal polyposis.

Hearing sensitivity in adults with a unilateral cleft lip and palate after two-stage palatoplasty.

OBJECTIVE: To evaluate long-term hearing and middle ear status in patients treated for a unilateral complete cleft lip and palate (UCLP) by two-stage palatoplasty. METHODS: Forty-nine UCLP patients aged 17 years and older were included in this retrospective study. Patients were invited for a multidisciplinary long-term follow-up of their treatment at a tertiary center for craniofacial surgery in the Netherlands. ENT assessment included tympanometry and pure-tone audiometry. Medical files were searched for medical and surgical history. RESULTS: In total, 19.4% of the patients had significant long-term hearing loss (PTA > 20 dB), comprising conductive hearing loss in 21.5% of the patients. In the majority (70%), this hearing loss was more pronounced at higher frequencies. In 25% the high fletcher index showed hearing thresholds above 20 dB. Ventilation tubes were placed at least once in 78.7% of the patients. The frequency of tube insertion was positively correlated with the incidence of reduced tympanic compliance (tympanogram type B) and the need for a pharyngoplasty. CONCLUSION: The present study reports long-term hearing outcomes in UCLP patients with hard palate closure at 3 years of age. Persistent hearing loss was observed in 19.4% of our patients (PTA > 20 dB), mainly at the higher frequencies. Ventilation tube placement was associated with reduced tympanic compliance and higher risk on hearing loss (PTA air conduction). Our high incidence of velopharyngeal incompetence during early childhood, which is likely associated with Eustachian tube dysfunction, might have contributed to these results.

Association of Iron Deficiency Anemia With Hearing Loss in US Adults.

Importance: Hearing loss in the US adult population is linked to hospitalization, poorer self-reported health, hypertension, diabetes, and tobacco use. Because iron deficiency anemia (IDA) is a common and easily correctable condition, further understanding of the association between IDA and all types of hearing loss in a population of US adults may help to open new possibilities for early identification and appropriate treatment. Objective: To evaluate the association between sensorineural hearing loss (SNHL) and conductive hearing loss and IDA in adults aged 21 to 90 years in the United States. Design, Setting, and Participants: The prevalence of IDA and hearing loss (International Classification of Diseases, Ninth Revision codes 389.1 [SNHL], 389.0 [conductive hearing loss], and 389 [combined hearing loss]) was identified in this retrospective cohort study at the Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania. Iron deficiency anemia was determined by low hemoglobin and ferritin levels for age and sex in 305 339 adults aged 21 to 90 years. Associations between hearing loss and IDA were evaluated using χ2 testing, and logistic regression was used to model the risk of hearing loss among those with IDA. The study was conducted from January 1, 2011, to October 1, 2015. Main Outcomes and Measures: Hearing loss. Results: Of 305 339 patients in the study population, 132 551 were men (43.4%); mean (SD) age was 50.1 (18.5) years. There was a 1.6% (n = 4807) prevalence of combined hearing loss and 0.7% (n = 2274) prevalence of IDA. Both SNHL (present in 26 of 2274 individuals [1.1%] with IDA; P = .005) and combined hearing loss (present in 77 [3.4%]; P < .001) were significantly associated with IDA. Logistic regression analysis confirmed increased odds of SNHL (adjusted odds ratio [OR], 1.82; 95% CI, 1.18-2.66) and combined hearing loss (adjusted OR, 2.41; 95% CI, 1.90-3.01) among adults with IDA, after adjusting for sex. Conclusions and Relevance: Iron deficiency anemia was associated with SNHL and combined hearing loss in a population of adult patients. Further research is needed to better understand the potential links between IDA and hearing loss and whether screening and treatment of IDA in adults could have clinical implications in patients with hearing loss.

Relation of otosclerosis and osteoporosis: A bone mineral density study.

OBJECTIVE: Otosclerosis and osteoporosis are metabolic bone diseases. In this paper, we investigated presence of osteoporosis with bone mineral density test in patients who had surgery for otosclerosis. METHODS: We included 27 patients who had conductive hearing loss and diagnosed with otosclerosis during an exploratory tympanotomy, and 30 healthy controls into this study. Bone mineral densitometry test was used for analysis of osteoporosis. T-scores of the patients and the controls obtained from L1-L4 vertebrae and femur neck were compared. In addition, the relations of duration of the disease, air and bone conduction thresholds, and air-bone gap in the operated ear with 25(OH)D3 levels, T-scores obtained from L1-L4 vertebrae and femur neck were investigated in the otosclerosis group. RESULTS: T-score obtained from L1-L4 region was -1.14±1.05 in the otosclerosis group, and was -0.56±1.10 in the control group; the difference was statistically significant (p=0.045). The T-scores obtained from the femur neck were -0.59±0.82 and 0.03±0.74 in the otosclerosis and the control groups respectively, with a statistically significant difference in between (p=0.004). As the air and bone conduction thresholds and the air-bone gaps of the patients with otosclerosis increased, 25(OH)D3 levels and T-scores decreased, but the differences were not statistically significant. CONCLUSION: The relation between otosclerosis and osteoporosis was shown with bone mineral density test in our study. T-scores of the otosclerosis patients were found smaller than the normal population.

Stapes surgery: a National Survey of British Otologists.

To investigate individual stapes surgery practice in the UK, a retrospective study was conducted by postal questionnaire to all 'assumed' stapes-performing otologists. 225 questionnaires were sent out to practicing otologists in the UK. 184 replies (81.8 %) indicated that 134 (72.9 %) otologists perform stapes surgery [stapedectomy (8.2 %), stapedotomy (91.0 %) or other (0.8 %)]. The '6-10 stapes operation per year' category is the most common, with most using general anaesthetic (GA) (78.3 %). Unilateral surgery is advised in 89.6 %, and 96.3 % perform second-side surgery, with all advising the option of a hearing aid prior to surgery. The majority (88.1 %) would fit the prosthesis after removing the stapes, with the top three prostheses being Causse, Smart and Teflon (as described by respondents). 42.5 % always use a vein graft or fat to cover the fenestration, 9.3 % use a laser and 48.5 % carry out the surgery as a day case. For an overhanging facial nerve (less than 50 % of the footplate obscured), the majority stated that it would depend whether they would abandon surgery. 25.4 % have encountered a 'gusher' and 83.6 % would recommend revision surgery. 82.8 % have a registrar present when carrying out stapes operations, but 69.4 % only offer training to trainees with an otological interest. In the UK, stapedotomy is the preferred technique. Most prefer the Causse prosthesis, general anaesthesia and an inpatient stay. Hearing aids are advised prior to surgery. Day-case and inpatient practice is about equal. 'Gushers' are encountered rarely. Revision surgery is advised if a conductive loss returns. Flying is recommended from 6 weeks. Most otologists are willing to teach trainees with an otological interest.

Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia.

PURPOSE: The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution. METHODS: We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p≤0.05). RESULTS: A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p=0.0130), prolonged course of hospitalization (p=0.0011), duration of mechanical ventilation (p=0.0046), requirement for tracheostomy (p=0.0013), and duration of loop diuretic (p=0.0005) and aminoglycoside therapy (p=0.0003). CONCLUSIONS: We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language.

The otolaryngologic manifestations of Sotos syndrome.

OBJECTIVE: Soto's syndrome is a genetic disorder caused by mutations in the NSD1 gene. It is characterized by excessive growth in early life. It features craniofacial abnormalities, developmental delay, hypotonia and advanced bone age. A review of the current literature reveals only chronic otitis media and conductive hearing loss as otolaryngologic manifestations of Soto's syndrome. Our objective was to determine if there are additional manifestations relevant to the otolaryngologist. METHODS: We performed a retrospective case series in which the Department of Defense electronic medical record was searched for ICD 9 code 253.0 (acromegaly/gigantism). Records were reviewed for genetic testing indicative of Soto's syndrome. These records were further analyzed for evidence of otolaryngologic problems. RESULTS: Seventeen patients were identified with five having confirmed NSD1 mutations consistent with Soto's syndrome. Of these, 4/5 had otolaryngologic problems such as conductive hearing loss, aspiration, laryngomalacia, obstructive sleep apnea and sensorineural hearing loss. CONCLUSIONS: Currently there is no description in the literature of these additional manifestations of Soto's syndrome. We present this case series to support the idea that an otolaryngologist should be involved in the multidisciplinary care required for these patients.

Otologic and audiologic characteristics of children with skeletal dysplasia in central China.

CONCLUSION: Otologic and audiologic abnormalities are both highly prevalent in Chinese children with skeletal dysplasias. Audiologic and otologic evaluations with medical intervention are recommended for these children. OBJECTIVE: Children with skeletal dysplasia have a number of otolaryngologic issues including hearing loss. This study aimed to investigate the otologic and audiologic features of Chinese children with skeletal dysplasia in central China. METHODS: Otologic evaluations and pure tone audiometry were performed in children with skeletal dysplasias. To explore the potential associations with hearing loss, we studied various craniofacial anomalies. RESULTS: A total of 125 children (mean age 9.2 years, range 5-18 years) with skeletal dysplasia were enrolled, and otoscopic examination identified eardrum abnormalities in 45.6% of the children. Audiologic analysis revealed 14 cases (11.2%) with conductive hearing loss (CHL) and 22 cases (17.6%) with sensorineural hearing loss (SNHL).

Age of diagnosis and evaluation of consequences of submucous cleft palate.

OBJECTIVES: To evaluate the frequency of submucous cleft palate (SMCP) in a group of children with clefts. The reason for suspecting submucous cleft, age of diagnosis, effect of age on speech development, problems in speech, hearing and swallowing were compared with previous literature. METHODS: Retrospective chart review: Out of 33 patients with SMCP, registered by the Groninger cleft team over approximately 20 years (1990 until July 2012), 28 non-syndromic patients with a proven diagnosis of SMCP were included: 17 males and 11 females. Speech and hearing were examined and the number of patients with SMCP and age at time of diagnosis were evaluated. The percentages of problems in resonance, articulation and hearing, present at time of diagnosis, were compared with the percentages of problems found after surgery. RESULTS: Out of 800 patients with clefts, 28 patients (3,5%) were diagnosed with SMCP at a mean age of 3;9 years. All patients presented one or more symptomatic complaints at time of diagnosis: hypernasality (65%), problems in articulation (46%), conductive hearing loss (39%) and/or swallowing problems (32%). A bifid uvula was found in 92%. Following surgery, hypernasal speech and swallowing problems were no longer observed. The articulation problems remained after surgery. Age of diagnosis seems no predictor of articulation problems. An improvement in hearing was observed but normal hearing was not achieved. Pharyngoplasty appeared to be a successful and save treatment of hypernasality. CONCLUSIONS: SMCP is a rare cleft palate which is, despite the presence of a bifid uvula and symptoms of velopharyngeal insufficiency, often diagnosed late. In children with a bifid uvula and mild problems in speech, hearing and swallowing, it is important to be alert to SMCP because SMCP may account for these persistent mild complaints. Therefore, early detecting of SMCP can yield profits.

[Measurement of quality of life following stapes surgery]. / Lebensqualitätbewertung nach stapesoperationen.

BACKGROUND: The objectives of this study were to compare a German version of the Glasgow Benefit Inventory (GBI) with the original English version and to describe quality of life following stapes surgery. PATIENTS AND METHODS: Audiometry and a questionnaire on handicap in various listening situations were used to assess 36 patients with otosclerosis before and 6 months after stapes surgery. The GBI was used to estimate the change in quality of life following surgery. RESULTS: Postoperative air-bone gap closure was <10 dB in 71% of patients. The postoperative air conduction threshold was <30 dB in 48% of patients. Using the GBI, the mean benefit score was 28 and the general benefit score 42. The social support and physical health scores were both zero. Where the postoperative improvement in air conduction was >15 dB, a significant reduction in handicap in everyday life, watching TV, listening to the radio and making telephone calls was seen. CONCLUSIONS: The German version of the GBI showed an improvement in quality of life in various everyday situations following stapes surgery.

Hearing loss due to wax impaction.

BACKGROUND: Deafness and hearing impairment are major causes of disability in developing countries. The World Health Organization (WHO) has pointed out that much deafness and hearing impairment is avoidable or remediable. A good proportion of patients presenting in the Ear, Nose, Throat, Head and Neck (E,N,T,H &N) Surgery Clinic of the University of Benin Teaching Hospital (UBTH),Benin City, have ear wax impaction causing hearing loss. OBJECTIVE: This study was therefore carried out to determine the prevalence of wax impaction among the aetiological factors for hearing loss, the type and severity of hearing loss due to wax impaction in our hospital. METHODS: Patients who met the inclusion criteria for the study were enrolled and Pure Tone Audiometry (PTA) threshold for each ear with wax impaction was determined at 250Hz, 500Hz, 1KHz, 2KHz, 4KHz and 8KHz by air conduction, Also bone conduction measurements were obtained at 500Hz, 1KHz, 2KHz and 4KHz. RESULTS: Forty-two out of 471 ears with hearing loss had wax impaction in this study; giving a prevalence of 8.9% for wax impaction among the aetiological factors for hearing loss. The result showed that mild hearing loss was the average and predominant type of hearing loss in terms of severity. Pure tone audiograms showed conductive hearing loss in all the patients, while 8 out of the 42 ears had a super-imposed sensorineural component, which gave a mixed hearing loss forthe ears. CONCLUSION: Wax impaction is a relatively common cause of hearing loss, usually of the mild conductive type. Appropriate strategies should be put in place for its management.