[Myringoplasties for anterior tympanic perforations: Our surgical technique]. / Myringoplastie pour les perforations tympaniques antérieures: notre technique opératoire.

Anterior tympanic perforations are actually the most difficult to close and a number of different techniques exist. This article represents the author's surgical procedure for type I tympanoplasties (myringoplasties) for this kind of perforation using the tragal cartilage and the perichondrium after preparation (revival, cleaning) of the perforation edge with a laser. This technique does not use a skin incision of external auditory meatus, when this one is large.

[Reconstruction of bone defects in the earcanal with hydroxyapatite]. / Reconstruction des defects osseux du conduit auditif externe par ciment d'hydroxyapatite.

Traditional reconstruction methods of osseous defects within the tympanic frame, most often being the atticotomy, within the framework of chronic otitis media surgery, still do not produce stable or definitive results, usually due to displacement or partial lyse of the transplanted material (cartilage, bone ...). The reconstruction procedure with the aid of hydroxyapatite cement as presented by the authors, allows for a complete, stable and definitive reconstruction.

Esteem 2 middle ear implant: our experience.

INTRODUCTION: The Esteem is a totally implantable hearing system that uses piezoelectric technology. It is indicated in case of moderate to severe stable sensorineural hearing loss with a minimum discrimination score of 60% and a middle ear which is anatomically and functionally intact. Its two components (sensor and driver) are positioned and fixed in the mastoid cavity and coupled respectively to the incus body and capitulum with cement. STUDY DESIGN: Retrospective study. SETTINGS: Tertiary referral center. MATERIALS AND METHODS: Pure-tone average (PTA) gain in the implanted ear was calculated at 1, 2, and 4 KHz, word recognition score (WRS) gain at 50 dB SPL, and average WRS (AWRS) gain at 40, 55, and 70 dB SPL. WRS was also evaluated in silence and noise. All patients were asked to fill in the abbreviated profile of hearing aid benefit (APHAB) questionnaire pre- and postoperatively as well as an Esteem questionnaire. RESULTS: Thirteen patients received implants between May 2008 and April 2010. Five minor complications occurred (1 temporary partial facial palsy, 1 secondary healing difficulty, and 3 revision surgeries for poor and deteriorating functional results and progressive gain loss after use of a heart defibrillator). Two patients (15%) suffered major complications and their implants had to be removed 4 months postoperatively because of a Staphylococcus aureus wound infection. One patient underwent reimplantation 6 months later. Mean PTA gain was 25 ± 11 dB, mean WRS gain at 50 dB SPL was 64 ± 33%, and mean AWRS gain was 40 ± 20%. WRS in silence and with a signal-noise ratio of 10, 0, and -5 dB was 91 ± 11, 85 ± 14, 71 ± 19, and 64 ± 30%, respectively. The APHAB questionnaire revealed 84% of satisfaction improvement compared to the previous classic hearing aid. CONCLUSION: The totally implantable hearing device Esteem 2 can offer good functional and satisfaction results. Careful selection of patients is required, however, based on hearing tests, exclusion of middle ear ventilation problems, and CAT-scan middle ear anatomy. Specific surgical training and experience are also needed. The implant is safe and only associated with classic auditory implant complications.

[Ossiculoplasty with hydroxyapatite bone cement]. / Apport du ciment d'hydroxyapatite en ossiculoplastie.

OBJECTIVE: To analyze the results obtained with hydroxyapatite bone cement (HABC). PATIENTS: A total of 73 ossiculoplasties with HABC were evaluated. Ears were divided into 4 groups: Group 1: Reinforcement of the incudo-stapedial joint with HABC; Group 2: Reconstruction of an incus long process defect with HABC; Group 3: Partial ossicular reconstruction between stapes and malleus handle with HABC; Group 4: Reconstruction of stapes with mobile or fixed footplate with moderate or extensive incus long process erosion using a titanium piston glued to the incus remnant using HABC. RESULTS: The mean follow-up was 21 months. The percentage of postoperative average air-bone gap (ABG) < or = 20 dB for groups 1, 2, 3 and 4 were 100%, 95%, 83% and 91% respectively; for ABG < or = 10 dB: 90%, 71%, 50% and 50%. No complications related to HABC and extrusion occurred. CONCLUSION: Today, the use of HABC seems to help us improve our functional results and also to avoid extrusion. In our experience, ossiculoplastie with HABC seems to provide better and more stable functional results. HABC is safe and easy to use. The use of cement with or without biocompatible ossicular prostheses allows us to repair different types of ossicular defects whilst trying to conserve an anatomical and physiological ossicular chain. Reconstruction of the incus long process or incudo-stapedial joint defect with cement is preferable rather than using partial ossicular reconstruction with HABC.

Tinnitus and otosclerosis.

OBJECTIVE: To establish the origin of tinnitus in patients with otosclerosis. METHODOLOGY: We performed a literature review to identify the characteristics of tinnitus in the general population and among patients with otosclerosis. RESULTS: Characteristics of tinnitus among patients with otosclerosis differ from those among the general population, suggesting mechanisms other than those implicated in presbycusis. Considerable improvements in the prevalence of tinnitus following corrective surgery demonstrate the limited contribution of diffuse labyrinth lesions. Middle ear and possibly secondary inner ear dysfunction produced by stapes blockage appear to be major etiopathogenic factors in the development of tinnitus in patients with otosclerosis. CONCLUSION: The conductive hearing deprivation produced by otosclerosis is associated with limited and reversible modifications in the central auditory pathway that are linked to tinnitus.

[Evaluation of the efficiency of ERTL4/6 screening tests for language disorders for children with a 22q11 deletion]. / Etude de l'efficacité des epreuves de repérage de troubles de langage ERTL4/A6 sur des enfants porteurs d'une microdélétion 22q11.

OBJECTIVE: ERTL (Epreuves de Repérage de Troubles de Langage) tests have been designed as a screening test to detect rapidly language disorders. To evaluate the effectiveness of these tests, we have compared them to the form P (NEEL-P) tests for the examination of language in 6 children with a 22q11 microdeletion. MATERIALS AND METHOD: 6 children aged from 4 years and 4 months to 5 years and 4 months, 4 boys and 2 girls have been tested. These tests have been performed in two sessions lasting between 1 and 1 1/2 hour. RESULTS: They are consistent in both tests. ERTL has identified all the children with a disorder. CONCLUSION: These fast tests should be used more often to detect children requiring additional and more complex language and neuropsychological evaluations.

"Tumulus" stabilization of a total ossicular replacement prosthesis.

Functional failure of total ossicular replacement prostheses (TORP) is often due to secondary displacement, ranging from simple subluxation to prosthesis extrusion following recurrence of severe tympanic membrane retraction. Several surgical techniques have been proposed to stabilize a TORP, mostly using superimposed non-organic or resorbable heterologous materials. We describe a simple so-called "tumulus" surgical technique that limits prosthesis displacement and extrusion, regardless of the type of TORP, by using a few fragments of autologous cartilage that are always available and perfectly tolerated at no cost. Review of 31 cases treated by this technique did not reveal any cases of prosthesis displacement and only one case (3.2%) of prosthesis extrusion with audiometric results comparable to the best results reported in the literature.

Today's indications for cochlear implantation.

Today's indications for cochlear implantation. During the last twenty years, the indications for cochlear implants (CIs) extended significantly due to positive experience with CIs, improved CI technology, and safer surgery. This paper reviews the classical and emerging indications for CIs anno 2007. Providing a postlingually deaf adult with a unilateral CI has been the earliest indication and remains the standard indication. However, CIs are also indicated for prelingually deaf adults, and for children younger than one year old. Recently, CIs are also indicated for adults with residual hearing: when best aided sentence recognition scores in quiet are lower than 70%. CIs for patients with residual hearing sometimes imply the use of a bimodal CI; a device that stimulates the cochlea both electrically and acoustically. Another promising evolution is bilateral implantation. Nowadays, it has also become possible to place a CI in the malformed cochlea. When an auditory nerve is absent or when implantation failed despite a functional device, auditory brainstem implants can restore some form of hearing to the deaf.

[TRT: results after one year treatment]. / TRT: efficacité après un an de traitement.

INTRODUCTION: Tinnitus Retraining Therapy (TRT) (which aims to induce changes in the mechanisms responsible for transferring signals from the auditory system to the limbic and autonomic systems) is a method for treating Tinnitus and decreased sound tolerance. An individualised explanation of Jastreboff's neurophysiological model allows greater insight and motivation on the part of the patient. Previous studies have demonstrated that daytime TRT is effective. As sleep forms a significant component of the distress associated with Tinnitus however, we hypothesised that night-time TRT could represent a useful tool in the treatment of this disabling condition. MATERIAL AND METHODS: 46 patients were studied (30 male, 16 female). Patients were selected from an ENT outpatient clinic. Patients with significant psychological disability were excluded. Patients were reviewed twice by their doctor and 5 times by a therapist over 12 months. Treatment consisted of 8 hours nighttime white noise stimulation, at progressively increasing intensity. Although several objective assessments of response were undertaken, patients' subjective testimonies were considered a more accurate signal of success. RESULTS: In total, 80% of patients had a satisfactory response after 1 year of treatment. 20% had no response. Patients were subcategorised according to Jastreboff's categories as follows: 1. Tinnitus (n = 6), 100% improved; 2. Tinnitus with hearing loss (n = 16); 62% improved; 3. Hyperacusis (with or without Tinnitus) (n = 16), 88.5% improved; 4. Hyperacusis (with or without Tinnitus, exacerbated by noise) (n = 8), 75% improved. CONCLUSION: Tinnitus is a symptom rather than an illness, and TRT gives patients greater control, allowing re-integration of normal perception. Night-time TRT is an effective treatment for Tinnitus and decreased sound tolerance. It has the potential advantage over day-time TRT of rapidly improving sleep and decreasing use of sedative hypnotics, a secondary effect noted in the personal testimonies of our cohort of patients. Further studies are needed to confirm this advantage, in view of the significant risks associated with long-term use of benzodiazepines. When investigating therapies for Tinnitus, it is necessary to measure success in terms of quality of life, as it is to this that the patient attaches the most importance.

The Tutopatch graft for transcanal myringoplasty.

OBJECTIVE: This article describes the technique and the preliminary results of transcanal myringoplasty for small or midsized tympanic membrane perforations with the Tutopatch (Tutogen Medical, Inc., Alachua, FL, USA), a xenograft derived from bovine pericardium. METHODS: All procedures started with a Rosen incision. The meatal skin was loosened from the bone up to the fibrous annulus, which was dislocated out of the sulcus, and the tympanic membrane was elevated from the malleus handle. The Tutopatch was placed over the malleus handle, the annulus, and the denuded bone. RESULTS: Eleven patients were included, and follow-up ranged from 3 to 14 months. Ten of the eleven eardrums were successfully closed. One patient developed myringitis 2 months after the procedure. The average air-bone gap at 0.5, 1, 2, and 4 kHz, of all but one patient was less than 10 dB. CONCLUSION: When anatomically feasible, a transcanal myringoplasty with a Tutopatch graft yields similar audiological results compared to fascia temporalis, perichondrium or tragal perichondrium-cartilage, but reduces the surgical trauma, the complications of the autograft harvesting, and the negative cosmetic and psychological impact of this procedure.

[Pathophysiology of cholesteatoma]. / La physiopathogénie des cholestéatomes.

OBJECTIVE: To describe the development of cholesteatoma using current knowledge. METHOD: Review of the literature. RESULTS: Cholesteatoma describes a mass of keratin (skin) in the middle ear which consists of a perimatrix and matrix. There are at least three kinds of cholesteatoma in the middle ear one resulting from invagination (retraction's pocket), another from migration and the last one from congenital inclusion. Cholesteatoma needs three successive inflammatory phases, the first leading to a retraction pocket, the second leading to pathology of the epidermis and of the floor of the external auditory canal and the third is the actual phase of cholesteatoma with invasion and middle ear auto-destruction with bone resorption. In this last phase, many factors play a role, collagenasis, osteoclats, cytokines, NO, bacteria and their biofilm and rupture of the retraction pocket. CONCLUSION: Cholesteatoma is an inflammatory disease of the ear caracterised by bone resorption. Current research is starting to appreciate the important role the immune system plays in the pathophysiology of cholesteatoma.

Metastatic renal cell carcinoma to the temporal bone: case report.

We report an extremely rare case of metastasic renal cell carcinoma to the temporal bone which presented initially as a jugulotympanic paraganglioma. The clinical and radiological appearances were misleading.Investigations of concomitant high blood pressure revealed a tumour of the right kidney. Biopsy of the mastoid mass was histologically compatible with a metastasis from a clear cell renal carcinoma. The patient underwent a radical nephrectomy and local external radiotherapy to the head. He also received adjuvant treatment with interferon-_ and interleukin 2. The clinical presentation, the radiological and histological features, the patterns of spread, the treatment options and the prognosis of these tumours are discussed. A review of the literature confirms the extremely unusual occurrence of this localisation.

Rehabilitation of high-frequency hearing loss with the RetroX auditory implant.

The RetroX is a new semi-implantable hearing aid which does not occlude the external ear canal. It consists of an electronic unit that plugs into a titanium tube which is implanted under the pinna so as to connect the retroauricular sulcus with the inside lumen of the external ear canal. Implantation requires minor surgery which can be performed under local anesthesia. Moreover, a pre-implant simulator exists and allows patients to try the device before deciding on definite implantation. The RetroX auditory implant is indicated in case of high-frequency sensorineural hearing loss with a steep slope (ski-slope audiogram). We implanted 25 adults suffering from such a hearing loss, and we report their hearing measured after 2.5 to 15 months of use. Four patients developed a persistent granulomatous reaction which disappeared after explantation. Two patients complained of acoustic feedback and needed supplementary fitting. Twenty three of our 25 subjects are satisfied or even extremely satisfied with the hearing improvement provided by the RetroX; they wear the implant daily, from morning until evening. Audiometrically, we observed a statistically significant improvement of the pure-tone thresholds at 1, 2, 4 and 8 kHz. In quiet, the speech reception thresholds decreased by 10 dB SPL and in noise, speech intelligibility increased by 15% for signal-to-noise ratios between -5 dB and +5 dB. Up till now, our patients were implanted monaurally because of financial considerations and our initial inexperience with this new implant. The overall results, however, are promising and nowadays, we advise bilateral implantation for patients who tolerate the first implant. By doing so, we hope to improve hearing in noise and spatial sound perception.

No evidence of auditory dysfunction in guinea pigs immunized with myelin P0 protein.

Recent data have focused on the peripheral nerve myelin glycoprotein P0 as a putative autoantigen involved in the autoimmune etiology of some cases of Meniere's disease, idiopathic sensorineural hearing loss and sudden deafness. To determine whether antibodies to myelin P0 can alter cochlear function, 13 healthy guinea pigs were immunized with purified porcine myelin P0 while 10 controls were injected with saline water. The animals were then evaluated for evidence of evolving inner ear disease using immunological, electrophysiological and morphological methods. Twenty-six experimental ears were tested weekly with a brainstem auditory evoked potential technique for a period of 4 months and were compared to 20 control ears. Uniformly, all P0-sensitized guinea pigs showed antibodies to myelin protein P0 as evidenced by ELISA. Clinical signs of inflammatory demyelination were not discernible in P0-sensitized guinea pigs and all the animals were qualitatively normal. No significant increase of evoked potential thresholds was found in the P0-sensitized animals when compared to controls (P>0.05). Peak latencies of waves I, II, III, IV and V and inter-peak latencies in P0-sensitized guinea pigs did not significantly differ from those of controls (P>0.05). Histological sections of inner ear and peripheral nerves were free of disease in both groups. These findings indicate that the sole presence of antibodies to myelin P0 in the sera of guinea pigs or patients suspected of having autoimmune inner ear diseases is unlikely to elicit auditory abnormalities and that additional factors are necessary for the pathogenic development of these disorders.

Autoimmune sensorineural hearing loss in young patients: an exploratory study.

OBJECTIVES: P0 protein is expressed exclusively in myelinating Schwann cells of the peripheral nervous system. In a previous study from our laboratory, 27% of patients with sensorineural hearing loss (SNHL) had antibodies to P0 protein in their serum. The purpose of the present exploratory study was to examine the relationship between the clinical presentation of SNHL among children and young adults (age range, 5-30 y) and the presence of serum anti-P0 antibodies. STUDY DESIGN: The data were collected by retrospective questionnaires from Belgian otolaryngologists. METHODS: Patients were divided for comparison into two groups according to the presence or absence of anti-P0 antibodies. RESULTS: Analyses of clinical data and audiometric results indicated that a progressive hearing loss was more frequently recorded in the patients in the anti-P0 antibody-positive group (82% [14 of 17]) than in those in the anti-P0 antibody-negative group (35% [6 of 17]) (P <.005). CONCLUSIONS: Thus, in the age group in the present study, autoimmune SNHL (as measured in the present study by the presence of anti-P0 antibodies) is more frequently associated with progressive than with sudden hearing loss. The implications of this finding for preventive screening of hearing loss in children and young adults are discussed.

Guinea pig inner ear antigens: extraction and application to the study of human autoimmune inner ear disease.

In this study, the authors attempted to develop a method of extracting guinea pig inner ear antigens for otoimmunological research, and to investigate the distribution of the antigens in the various structures of the inner ear. The antigens were extracted either from the entire or from various parts of the guinea pig inner ear. These antigens were separated on sodium dodecylsulfate-polyacrylamide gel electrophoresis (SDS-PAGE) gels. Western blot techniques were used to test sera from patients with inner ear disease against guinea pig inner ear protein extracts. It found that the various molecular weight antigens in the inner ear were associated with the different structures of the inner ear. The sera of 37.5% (N = 80) of patients reacted with two bands (30 and 58 kd) of the guinea pig inner ear immunoblots. The 58 kd band was not specific to the inner ear, but instead was also found in the immunoblots of other guinea pig tissues (brain, lung, and liver). This study suggests that the various antigens of interest could be further extracted and purified from the corresponding locations of the inner ear.

COCH5B2 is a target antigen of anti-inner ear antibodies in autoimmune inner ear diseases.

OBJECTIVE: This study was designed to identify the 58-kDa inner ear protein against which the sera of some patients with idiopathic, progressive sensorineural hearing loss or Ménière's disease strongly react. BACKGROUND: We and other groups have previously demonstrated that a 58-kDa protein extracted from guinea pig or bovine inner ear tissue is a target of antibodies in serum samples from some patients with autoimmune inner ear diseases. METHODS: After separation of inner ear proteins by 10% sodium dodecyl sulfate polyacrylamide gel electrophoresis, the bands corresponding to 58 kDa were localized and excised from the gel. The concentrated protein was then digested with trypsin, and the peptide fragments were separated by high-pressure liquid chromatography. Three fractions were subjected to amino acid sequencing by the classic Edman degradation. RESULTS: The sequence of a stretch of 14 amino acids of the first fragment was identical to that of amino acids 526 to 539 of the COCH5B2 protein. The sequences of 11 and 10 amino acids of the second and third fragments, respectively, also were identical to residues 417 to 427 and 396 to 405 of the COCH5B2 protein. These data, together with two-dimensional gel electrophoresis followed by Western blot experiments, confirmed that the 58-kDa inner ear protein is the COCH5B2 protein. DISCUSSION: These findings indicate that the 58-kDa target protein of antibodies in serum samples of patients with autoimmune inner ear diseases is the COCH5B2 protein, a molecule that is highly and specifically expressed in the cochlea and vestibule.

Macroporous biphasic calcium phosphate efficiency in mastoid cavity obliteration: experimental and clinical findings.

Following our previous experimental studies on the performance of macroporous biphasic calcium phosphate (MBCP) in canine mastoid cavities, we used this material in patients requiring surgical intervention. Twenty-two cases were selected, and in eight specific cases a biopsy specimen was taken. Histologic, ultrastructural, and microanalysis studies were performed. This study demonstrates the effectiveness of MBCP implants as bone graft substitutes for mastoid cavity obliteration. Clinical evaluation of the series and histologic and ultrastructural results demonstrated the bioactivity and osteo-conduction of this material, with partial transformation of MBCP granules into lamellar bone after several months.

HLA class II-associated genetic susceptibility in idiopathic progressive sensorineural hearing loss.

To investigate the association between genes in the major histocompatibility complex and inner ear disease susceptibility at the DNA level, high-resolution genotyping for HLA class II (HLA-DR, -DQ, -DP) was performed by polymerase chain reaction-sequence-specific oligonucleotide reverse dot blot and polymerase chain reaction-restriction fragment length polymorphism analysis in 34 patients with idiopathic progressive sensorineural hearing loss (PSHL) and in 214 controls. The frequencies of DRB1*0301, DRB3*0101, DQB1*0201, and DPB1*0401 were significantly increased in patients with idiopathic PSHL compared with controls. The DQB1*0301 allele was significantly decreased in the patients. A linkage disequilibrium was probably responsible for the concomitant increase of both DRB1*0301 and DRB3*0101 alleles in patients. The increase of DQB1*0201 in patients was associated with the DRB1*0301 allele. In addition, the telomeric DPB1*0401 allele may act as an independent risk factor. The DQB1*0301 allele may have a protective role in the pathogenesis of idiopathic PSHL. These results suggest that the specific HLA class II gene products may confer susceptibility or resistance to idiopathic PSHL.

Inner ear autoantibodies and their targets in patients with autoimmune inner ear diseases.

Immunological mechanisms are thought to play an important role in the pathogenesis of some cochleo-vestibular diseases. This study attempts to present further evidence of autoantibodies reactive against guinea pig inner ear proteins found in patients with autoimmune inner ear diseases (AIED) and specifically identifies the main target antigens of these antibodies. Sera from 110 patients with a clinical diagnosis of either rapidly progressive sensorineural hearing loss (n = 32). Ménière's disease (n = 41), sudden deafness (n = 6) or other aetiologies of hearing loss (n = 11) were screened by the Western blot technique. Forty-four percent of the patients' sera had antibodies to several inner ear proteins, of which the 30, 42 and 68 kDa proteins were found to be the most reactive. These highly reactive proteins were identified by gas-phase micro sequencing after digestion with trypsin and separation of peptide fragments by high-performance liquid chromatography. A partial sequence of each protein was determined. These data, together with those obtained from 2-dimensional gel electrophoresis followed by Western blotting, demonstrated that the 30 and 42 kDa inner ear proteins are the major peripheral myelin protein P0 and the beta-actin protein, respectively, while sequence analysis indicated that the 68 kDa protein is novel. These findings further support the hypothesis that several populations of antibodies may contribute to the enhanced immunological activity of AIED patients. They also add a new dimension to our knowledge of AIED and may open new avenues in the development of simple serological assays, which are easier to perform and more rapid than Western blotting.