Hearing loss due to mannosidosis and otitis media with effusion. A case report and review of audiological assessments in children with otitis media with effusion

A case of a child with mannosidosis and bilateral otitis media with effusion (OME) is reported here along with some discussion of relevant literature to emphasize the need for age appropriate audiometric assessment before and after insertion of grommets for glue ear (OME). There is a need for multidisciplinary teamwork in the management of children with hearing loss. If OME is treated surgically, age-appropriate hearing assessment is required before and after insertion of grommets. The need for audiological assessments will be relevant even if children had passed the newborn hearing screening test.     

Chronic otitis media with effusion sequelae in children treated with tubes

OBJECTIVE: To determine incidence and prevalence of middle ear sequelae and abnormal tympanometry results among children with chronic otitis media with effusion (OME) who received standard treatment with tympanostomy tubes. DESIGN: Prospective cohort study. SETTING: Community clinic and academic medical center.Patients A total of 140 children followed up for 8 years after tube treatment. MAIN OUTCOME MEASURES: Tympanic membrane perforation, atrophy, retraction, hearing loss, myringosclerosis, low static admittance (SA) and broad-peaked tympanogram, high SA and narrow-peaked tympanogram, and negative tympanometric peak pressure. RESULTS: Annual incidence of sequelae was typically greater during 3 to 5 years than 6 to 8 years of follow-up. Greatest increases in incidence during the 5-year follow-up were for atrophy (67%), high SA and narrow-peaked tympanogram (70%), and retraction pocket (47%). Prevalence of these sequelae also increased over time, whereas low SA and broad-peaked tympanogram and negative tympanometric peak pressure decreased during follow-up. Sequela tended to become bilateral over time, and concordance of different sequelae in the same ear was low (kappa, 0.05-0.42). CONCLUSIONS: Annual incidence of sequelae decreased during follow-up. This finding parallels decreasing incidence of OME and tube placement as children mature and demonstrates that sequelae are more likely to develop during active acute and chronic OME. The cumulative effect of incidence resulted in few ears free of sequelae by 8 years of follow-up. Based on this cohort of healthy children with OME, although the risk of sequelae decreased over time, functional and morphologic sequelae were prevalent and may put children at risk for continuing middle ear problems as they grow into adolescence and adulthood.     

Clinical outcomes of otitis media with effusion following palatoplasty in patients with incomplete cleft palate

Background: Diagnosis and treatment strategies for CP with OME and the timing of ear tube insertion remain controversial.

Objectives: To analyze the clinical outcomes of otitis media with effusion in children with incomplete cleft palate after palatoplasty prospectively.

Methods: A total of 30 children (10 months–2 years old) with incomplete CP were enrolled in this study and received at least 6 months of follow-up evaluations after palatoplasty.

Results: The overall improvement rate of OME was as high as 26% in this group. Average air conduction hearing threshold was significantly better than that before surgery in the 8 patients with improved OME (p?<?.05). Among the 8 children with improved OME, 7 (87.5%) were found to have middle ear effusion that improved within 6 months after CP repair. There was no significant difference in the improvement rate between the severe degree II group and the mild group.

Conclusion: A 6-month follow-up period is recommended. The severity of incomplete CP is not fully related to the function of the eustachian tube.

Significance: The overall improvement rate was as high as 26%, and effusion in the tympanic cavity subsided in 7 out of 8 cases within 6 months after the CP repair.     

Relationships between otitis media sequelae and age

Objectives: To explore relationships between age and sequelae in two groups of children treated with tympanostomy tubes for chronic otitis media with effusion (OME). Study Design: Cross-sectional study of sequelae among children, adolescents, and adults at 4 years and 9 to 23 years after tympanostomy tube treatment. Methods: Group I was examined with otomicroscopy, tympanometry, and audiometry two to four times a year as part of a prospective study, and they were evaluated 4 years after initial tube treatment for this study. Group II received tubes while participating in a chronic OME study, but participants were not followed prospectively after treatment. Nine to 23 years after tube treatment, they were examined with otomicroscopy, tympanometry, and hearing screening. Results: Among the 5? to 28? year-old subjects, cholesteatoma (?1%) and perforation (?2%) were rare. In Group I, tympanosclerosis increased with age (P < .01), and OME (flat tympanograms) decreased with age in Group II (P < .01). The older cohort was more likely to have severe retractions (18% vs. 4%, P = .02), hearing loss (21% vs. 10%, P < .01), and severe atrophy (24% vs. 0%, P < .01) than the younger cohort, but they were less likely to have flat tympanograms (2% vs. 12%, P < .01). Conclusions: Although OME became less prevalent with age, important sequelae (severe atrophy, severe tympanic membrane retraction, hearing loss, cholesteatoma, and chronic perforation) may develop in children with chronic OME as they become adolescents and young adults. Long-term prospective studies are important in defining the progression of sequelae in these children.     

A longitudinal evaluation of hearing and ventilation tube insertion in patients with primary ciliary dyskinesia

IntroductionPrimary ciliary dyskinesia (PCD) is an autosomal recessive genetic disease, which primarily manifests with oto-sino-pulmonary symptoms. Otitis media with effusion (OME) is common from early childhood. The existing literature on OME management in PCD is conflicting.The goals of the present study were firstly to evaluate the long-term hearing in PCD patients and secondly to determine the influence of ventilation tube (VT) insertion on hearing and postoperative otorrhoea.MethodsA longitudinal retrospective study extracting the hearing level (pure tone average (0.5, 1, 2, 4 kHz, PTA)) and tympanometry from the medical records. Furthermore, the patient files were reviewed for VT insertion and postoperative otorrhoea. Postoperative otorrhoea was defined prolonged when it lasted for four weeks or longer.ResultsFifty-seven patients were identified in a 30-year period, age 2–72 years, and 278 audiometries were evaluated. The median number of audiometries per patient was 3 (range 1–29) and the median follow-up was 60 months (range 0–351 months). The mean PTA was 34 dB HL in patients below six years of age and improved significantly (p < 0.0001) with age. VT insertion improved hearing; however, 48% of patients with VT insertion experienced prolonged otorrhoea.ConclusionIn this study of PCD patients the hearing improved as a function of age as well as following VT insertion. However, VT insertion was also associated with prolonged otorrhoea.     

Auditory processing abilities in children with chronic otitis media with effusion

Conclusion The study results indicate that children with a history of otitis media with effusion (OME) suffer from auditory processing disorder to some degree. The findings support the hypothesis that fluctuating hearing loss may affect central auditory processing during critical periods.

Objectives Evidence suggests that prolonged OME in children can result in an auditory processing disorder, presumably because hearing has been disrupted during an important developmental period. A lack of auditory stimulation leads to the abnormal development of the hearing pathways in the brain. The aim of the present study was to determine the effects of OME on binaural auditory function and auditory temporal processing.

Method In the present study, the dichotic digit test (DDT) was used for binaural hearing, and the gap in noise (GIN) test was used to evaluate temporal hearing processing.

Results The average values of GIN differed significantly between children with a history of OME and normal controls (p?p?=?0.002).     

Parental perceptions and understanding of information provision,management options and factors influencing the decision-making process in the treatment of children with glue ear

ObjectivesOtitis media with effusion (OME) is a common cause of hearing loss and possible developmental delay in children, and there are a range of ‘preference sensitive’ treatment options. We aimed to evaluate the attitudes and beliefs of parents of affected children to treatment options including watchful-waiting, hearing aids, grommets, and, oral steroids with the intention of developing our understanding of decision-making and the factors influencing it, sources of parental information, and satisfaction with information provision.DesignWe recruited a convenience sample of twelve parents of eleven children with OME at a single ENT department of a teaching hospital into a qualitative research study. The children of the parents interviewed had already been recruited into the Oral Steroids for the Resolution of Otitis Media with effusion In Children (OSTRICH) study. Semi structured interviews were audio recorded, transcribed and then coded using an inductive, thematic approach.ResultsParents were satisfied with the verbal provision of information during the treatment consultation, although many were keen to receive supplementary printed information. Discussion with family and friends helped the decision-making process, whereas insufficient information and a paternalistic approach were viewed as obstacles. Parents were particularly influenced by the following: the immediacy of the treatment option effect, perceived efficacy, perceived risks and adverse effects, social implications (especially with hearing aids) and past personal and informant experience.ConclusionsParents appreciate clinicians tailoring information provision to parents' information needs and preferred format. Clinicians should also elicit parental attitudes towards the different management options for OME and the factors influencing their decisions, in order to optimise shared-decision making and ultimately provide a better standard of clinical care.     

Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate1

Otitis media with effusion (OME) is common among children with cleft palate, and may lead to such long‐term consequences as hearing loss, tympanic membrane retraction, and chronic otitis media (COM). In total, 104 children with cleft lip and/or palate treated for OME at our institution were reviewed. Mean duration of follow‐up was 6.9 years, and mean age at latest follow‐up was 9.6 years. The incidence of COM was 19%, and the incidence of cholesteatoma was 1.9%. Ears showing such long‐term sequelae of OME as hearing loss, tympanic membrane retraction, and chronic otitis media, were noted to have undergone a significantly greater number of ventilation tube insertions than ears not showing these sequalae. Our findings would suggest that a conservative approach to the management of OME in children with cleft palate is more likely to be beneficial in the long term.     

Does the use of radiofrequency myringotomy for insertion of a ventilation tube reduce the incidence of myringosclerosis?

The aim of the present study was to determine the rate of myringosclerosis after radiofrequency (RF) myringotomy and ventilation tube (VT) insertion and compare it with that after the incisional myringotomy and VT insertion. Thirty children (60 ears), 2–16 years old (mean age 7.06 ± 2.77 years) who were planned to undergo surgical intervention for bilateral otitis media with effusion (OME), were included in this study. The children were treated by RF myringotomy of the right ear, incisional myringotomy of the left ear, and insertion of VTs into both ears. Both ears were examined intraoperatively for bleeding, and patients were evaluated for myringosclerosis formation with otomicroscopy at the end of the ninth month. Myringosclerosis was observed in 22 of the 60 ears. The overall incidence was 36.6 %. Fifteen (50 %) left ears showed myringosclerosis by otomicroscopy, and seven (23.3 %) right ears showed myringosclerosis. The rate of myringosclerosis of the right ear was significantly lower than that of the left ear (p < 0.05). In addition, intraoperative tympanic membrane bleeding was observed in 24 (40 %) of the 60 ears: 21 (70 %) left ears and three (10 %) right ears were perforated by RF. The tympanic membrane bleeding rate of the right ear was significantly lower than that of the left ear (p < 0.01). The present study is the first to determine the myringosclerosis rate after RF myringotomy and VT insertion. Our results indicate that VT insertion with RF myringotomy decreased the incidence of myringosclerosis.     

Feasibility of hearing preservation for residual hearing with longer cochlear implant electrodes

Abstract

Background: Hearing preservation is thought to be achievable following atraumatic surgery with thin cochlear implant electrodes; therefore, the surgical approach and implant electrode design are crucial considerations.

Objective: To assess the feasibility of hearing preservation with long electrodes for patients meeting the criteria for conventional cochlear implantation.

Methods: One hundred and two patients (132 ears) who underwent cochlear implant surgery were analyzed. Inclusion criteria included measurable residual hearing in the low frequency before implantation and not meeting the criteria for electric acoustic stimulation (EAS).

Results: Of the 18 patients with residual hearing in the low frequency enrolled, 17 subjects (94.4%) retained low frequency hearing. A younger age at surgery tended to contribute to better hearing preservation than that observed in older patients. There was no clear trend regarding the influence of insertion depth angle of the electrode on hearing preservation.

Conclusion: It is possible to achieve hearing preservation in the lower frequency by the use of longer electrodes. This study underscores the importance of atraumatic surgery, even for patients with only limited residual hearing, and longer electrodes should be adopted for EAS.     

Clinical,surgical, and electrical factors impacting residual hearing in cochlear implant surgery

Abstract

Conclusions: This study recommends using soft surgical principal and round window insertion to protect residual hearing with favorable anatomical exposure. Further studies are needed to evaluate the impact of the electrical stimulation on the organ of corti and hearing.

Objective: The objective of this study is to analyze various factors that impact on preservation of residual hearing post-implantation.

Methods: A retrospective study was performed to analyze loss of residual hearing in a cohort of 225 patients implanted in a large academic center. Sixty-four patients met the inclusion criteria. The impact of age at implantation, gender, etiology of hearing loss, cochleostomy vs round window insertion, partial vs full insertion, and effect of initial stimulation were analyzed using appropriate statistical analysis.

Results: The overall hearing preservation rate for all implanted patients was 64%. Loss of residual hearing was significantly more observed in cases of cochleostomy and/or non-soft surgical techniques. No correlation was observed with age at implantation, gender, side of implant, device manufacturer, and presence of pre-lingual deafness vs post-lingual, full or partial electrode insertion. In addition, there was a small but significant decrease in hearing between pre-stimulation and post-stimulation audiograms at 6000 Hz.     

Expression of aquaporins mRNAs in patients with otitis media

Objective: This study analyzed the associations between measured levels of aquaporin (AQP) mRNAs and clinical manifestations in patients with various types of otitis media (OM).

Methods: AQP1, 2, 3, 4, 5, 6, 8, and 10 mRNA levels were assayed by real-time PCR from 57 patients with chronic otitis media (COM), 24 patients with cholesteatomatous otitis media (choleOM), and 82 patients with otitis media with effusion (OME). The relationships of these mRNA levels with the presence of bacteria, the type of hearing loss, and clinical manifestations of OM types were evaluated.

Results: All eight AQP mRNAs were expressed in inflammatory tissue, chole matrix, and effusion fluid obtained from all 163 patients with OM. The levels in OME of AQP2, 4, 6, and 10 mRNA; and the levels in choleOM of AQP1, 3, 4, and 10 mRNA were elevated significantly compared to the corresponding levels in COM (p?p?Conclusions: The levels of expression of AQP mRNA are associated with the pathophysiology of OM.     

Electric-acoustic stimulation with longer electrodes for potential deterioration in low-frequency hearing

Abstract

Background: Electric-acoustic stimulation (EAS) has emerged as a standard treatment for patients with high-frequency hearing loss. EAS is usually performed with shorter electrodes of 16–24?mm in length. As most EAS recipients gradually lose residual acoustic hearing in the implanted ear over time, EAS with longer electrodes without causing significant intra-cochlear damage might be ideal.

Objective: The aim of this study was to investigate hearing preservation (HP) results after EAS surgery with longer electrodes.

Methods: Ten patients (11 ears) with partial deafness that met the indications for EAS with a MED-EL FLEX28 electrode were included in this study. Auditory thresholds before and at 6?months after activation were examined.

Results: In 100% of cases, HP was comfortably achieved, indicating that all patients could utilize acoustic amplification combined with electric stimulation.

Conclusion: EAS with longer electrodes can offer broader cochlear coverage, resulting in natural frequency matching in comparison with shorter electrodes, even in EAS cases. The combination of advanced surgical techniques and flexible, long, straight electrodes permits deep insertion that reaches the apical region with little or no insertion trauma.     

Use of a mid-scala and a lateral wall electrode in children: insertion depth and hearing preservation

Conclusions: Atraumatic insertion of the HiFocus^TM Mid-Scala (HFMS) electrode via the round window was successfully achieved in seven children. Residual hearing 6 months post-operatively was preserved to within 10?dB HL of the pre-operative audiogram at 500?Hz for six children, indicating minimal initial insertion trauma to the cochlea.

Objectives: The objectives were to document the clinical experience and evaluate differences between HFMS and HiFocus^TM 1j (HF1j) by means of insertion depth and hearing preservation results.

Method: Nineteen children were prospectively recruited and consecutively implanted with the HF1j electrode (n?=?12) or the HFMS electrode (n?=?7) via the round window. Average median angular insertion depths and the amount of residual hearing preserved at 6 months post-operatively were compared between the two electrode groups.

Results: The median angular insertion depth for the HF1j was 439° and for the HFMS 435°. Preservation of residual hearing at 500?Hz was assessed in seven HFMS subjects and 11 HF1j subjects. Based on the Skarzynski formula, three out of seven subjects (42%) in the HFMS group had their residual hearing completely preserved at 500?Hz. In the control group, no subjects had complete hearing preservation and five subjects had a complete loss of residual hearing.     

Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate

Otitis media with effusion (OME) is common among children with cleft palate, and may lead to such long-term consequences as hearing loss, tympanic membrane retraction, and chronic otitis media (COM). In total, 104 children with cleft lip and/or palate treated for OME at our institution were reviewed. Mean duration of follow-up was 6.9 years, and mean age at latest follow-up was 9.6 years. The incidence of COM was 19%, and the incidence of cholesteatoma was 1.9%. Ears showing such long-term sequelae of OME as hearing loss, tympanic membrane retraction, and chronic otitis media, were noted to have undergone a significantly greater number of ventilation tube insertions than ears not showing these sequalae. Our findings would suggest that a conservative approach to the management of OME in children with cleft palate is more likely to be beneficial in the long term.     

Have two UK national guidelines had any effect on grommets day-case utilisation and rate over the last 10?years?

Grommets insertion is a common otolaryngological procedure for the treatment of persistent otitis media with effusion. In 2002, the Department of Health (DoH) Day Surgery guidelines stipulated that at least 75% of grommets insertions should be undertaken as day-cases. In 2008, after governmental perception of a higher than necessary grommet insertion rate, the National Institute for Health and Clinical Excellence (NICE) guidelines aimed at reducing inappropriate grommet insertions. This study analyses the effect these national interventions have had on grommets insertion in England and Wales. A retrospective study was undertaken. Data were extracted from the patient episode databases of England (Health Episode Statistics) and Wales (Patient Episode Database of Wales) from 2000 until 2010 using OPCS-4 code D151. Statistical change in practice following the introduction of the interventions was assessed using linear regression. Results: 341,526 and 16,400 grommets insertions were performed in England and Wales, respectively. Linear regression analysis demonstrated that implementation of the Day Surgery guidelines significantly improved day-case rates in both national cohorts (England P?<?0.0001, Wales P?<?0.0001) and reduced mean waiting times for grommets insertion in both cohorts (England P?<?0.05, Wales P?<?0.01). Regression analysis also showed that implementation of the NICE guidelines had no effect on the number of grommet insertions in England (P?>?0.5) and Wales (P?>?0.5). In conclusion, the DoH guidelines have increased grommets day-case provision and reduced waiting times in both England and Wales, whereas the NICE guidelines have not affected overall levels of grommet insertion in either national cohort.     

Myringosclerosis after tympanostomy tube insertion: Relation with tube retention time and gender

Objective

To determine relationship between myringosclerosis and tube retention time and sex in children with chronic otitis media with effusion who were treated with tympanostomy tube insertion. Also, the relationship between myringosclerosis both sex and initial age of tube insertion were investigated.

Methods

A total of 101 children (195 ears) were reviewed. Ears were divided into four groups according to retention time of tympanostomy tubes. Group I: Retention time of tympanostomy tube less than 6 months. Group II: Retention time of tympanostomy tube from 6 months to 12 months. Group III: Retention time of tympanostomy tube of 12 months or more. Group IV: Myringotomy group without tympanostomy tube insertion.

Results

The order of the myringosclerosis rates were as follows from the highest to lowest one; group III (44.1%), group II (42.4%), group I (14.3%), and group IV (7.7%). Myringosclerosis was more common in group I compared with group IV, but the difference was statistically not significant (p > 0.05). There were no statistically significant differences in myringosclerosis rates between the group II and group III (p > 0.05). On the other hand, statistically significant differences were observed in myringosclerosis rates between group I and group II (p < 0.05), and between group I and group III (p < 0.05); also similar significant differences were present in myringosclerosis rates between group IV and group II (p < 0.05), and between group IV and group III (p < 0.05). There was no significant difference between preschool age group and school age group. Myringosclerosis was observed in 40% of boys and in 51.2% of girls.

Conclusion

Myringosclerosis is frequent in patients who underwent tympanostomy tube insertion. The frequency of myringosclerosis is much higher in tympanic membranes with tympanostomy tube insertion than tympanic membranes with myringotomy, and the location of sclerotic plaques does not always correspond to the tympanostomy area. The myringosclerosis rate was increased when the tympanostomy tubes stay on tympanic membrane for a long time. Highest myringosclerosis rates were observed if the extrusion time was 12 months or longer. In our analysis, sex and initial age of tube insertion were not significant factors for the development of myringosclerosis after extrusion of tympanostomy tubes.     

Glue under pressure: a bad prognostic sign for recurrence of otitis media with effusion.

One hundred and thirteen children with bilateral otitis media with effusion (OME) underwent myringotomy and insertion of Shah grommets. They were classified into three groups according to the presence or absence of 'glue under pressure' unilaterally or bilaterally. The follow up period ranging between 18 and 32 months determined the comparative rate of recurrence of OME and the number of grommet reinsertions. This study shows a significantly higher incidence of recurrent OME, requiring grommet reinsertion, in ears with glue under pressure (60 per cent) compared to those with glue not under pressure (7.4 per cent). Thus it was possible to identify a subset of children with OME who have a poorer prognosis for recurrence and who should be treated with long-stay grommets in the first instance.     

Hearing acuity in otitis media with effusion and adhesive otitis media

The relationship between findings established by the photograph of the tympanic membrane and hypacusia was determined by pure-tone audiometry. Subjects were 352 ears of 267 patients diagnosed as otitis media with effusion (OME) or adhesive otitis media (AdOM). These two diseases were evaluated on the basis of photographs of the tympanic membrane taken from 1983 to 1988. The results were as follows; 1) The effect of aging process on hearing acuity was noted in patients with these diseases, i.e., values of hearing acuity by air and bone conduction were, in descending order, early-, mature-, and advanced-age groups, with significant differences (p less than 0.01). 2) Greater loss of acuity by air conduction in the middle to high frequency ranges was found in patients with OME than with AdOM. In bone conduction, a decrease in low frequency range in the early--and mature--age groups was larger in patients with AdOM than with OME and a significant decrease in 2000-6000 Hz in the advanced-age group was more marked in patients with OME than with AdOM (p less than 0.05). 3) Among patients with AdOM, those with retained fluid in the middle ear showed a significant decrease in air conduction values and a significant increase in air-bone gap compared with those without fluid (p less than 0.05). 4) The degrees of adhesion correlated well with a decrease in hearing acuity. 5) Hearing acuity was not influenced by attic retraction, white plaque, scar and atrophy of their tympanic membranes.     

Gastroesophageal reflux and sequelae of chronic tubotympanal disorders in children

Objective—To compare sequelae of chronic tubotympanal disorders in children with and without gastroesophageal reflux (GER).

Material and Methods—In 32 patients with chronic tubotympanal disorders GER was studied by means of 24-h continuous esophageal pH monitoring. After a period of 2–6 years (mean 4 years) sequelae of the tubotympanal disorders were examined, together with the clinical status of the ears and hearing status. The criteria for classification into mild, moderate and severe sequelae were based on the clinical status of the ear. Hearing was determined using tonal audiograms. Conductive hearing loss was classified as either slight (≤ 35 dB hearing loss in speech frequencies) or severe (>35 dB hearing loss in speech frequencies).

Results—In the group of 16 patients (mean age 6.1 years) with GER, sequelae were observed in 29 ears (mild, n=11; moderate, n=5; severe, n=13). Hearing impairment was determined in 20 ears (mild, n=8; severe, n=12). In the group of 16 patients (mean age 7.1 years) without GER, sequelae were observed in 17 ears (mild, n=11; moderate, n=1; severe, n=5). Hearing impairment was determined in 10 ears (mild, n=6; severe, n=4).

Conclusion—The total number of ears with sequelae and the total number of ears with conductive hearing impairment were significantly higher in patients with GER.