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The Lacrimo-Auriculo-Dento-Digital (LADD) syndrome: temporal bone CT findings.

The temporal bone CT examination of a 16-year-old female patient with the LADD syndrome or Levy-Hollister syndrome showed multiple bilateral middle as well as inner ear malformations. Ossicular chain anomalies were seen, especially of the incus and stapes. The oval window was very narrow to absent. Both cochleas were hypoplastic and showed modiolar deficiency. A common cavity between the vestibule and lateral semicircular canal was bilaterally present.

Abnormalities, Multiple↗

Radiologic and mechanical properties of inactivated ossicle homografts.

OBJECTIVE: This study examined the effects of old and new inactivation (sterilization) techniques on the radiologic and mechanical properties of ossicle homografts. MATERIALS AND METHODS: Ninety normal incuses and malleuses received either treatment with 1) 5% formaldehyde/cialit, 2) 1N NaOH, 3) 0.9% LpH, or 4) autoclaving at 134'C, or no treatment. All ossicles were assessed radiologically by high-resolution computed tomography. After imaging, all ossicles underwent mechanical testing by destructive axial compression in a mechanical testing machine measuring force and displacement. RESULTS: Ossicles treated with cialit, NaOH, or autoclaving showed a significant decrease of ultimate force and stiffness compared with controls. LpH treatment caused no such changes in these structural properties. Material properties of yield strength, ultimate strength, and elastic modulus were also altered by cialit, NaOH, and autoclaving, but were much more difficult to assess because of uncertainty in parameter estimates. There was a significant increase in radiologic density in autoclaved ossicles, a reduction in cialit- and LpH-treated ossicles, and no change in NaOH-treated ossicles. CONCLUSIONS: All tested inactivation procedures changed the biomechanical and/or radiologic properties of ossicle homografts. However, the new procedures used to inactivate infectious agents produced changes similar to the older treatments with formaldehyde/cialit. Human allografts are able to withstand harsh but safe sterilization procedures. The NaOH treatment seems to be the most suitable method for the future. The biologic (osteogenic) potentials of ossicle homografts treated with these new preservation/inactivation methods are still unknown. Further investigations are necessary to re-evaluate the clinical use of ossicle homografts in middle ear reconstructive surgery.

Adult↗

Surgical techniques to facilitate endoscopic second-look mastoidectomy.

OBJECTIVES/HYPOTHESIS: Mastoidoscopy has shown to be a safe, effective alternative to traditional second-look mastoidectomy. This study was undertaken to review surgical modifications to facilitate successful mastoidoscopy. STUDY DESIGN: Retrospective database review of all surgical procedures performed by the senior author (T.J.H.) since January 1995. All surgeries were performed in a tertiary hospital setting. RESULTS: Fifteen second-look procedures were performed in this series. Five were performed endoscopically, 10 with traditional techniques. In the traditional surgeries five were prior to the use of endoscopy, five had contraindications to endoscopic mastoidectomy. There were six residual cholesteatomas in the series, one in the endoscopic cases (20%), and five in the traditional cases (50%). No cholesteatomas were identified with microscopic examination performed after endoscopy. There were no complications in the series. Mastoidoscopy gives limited access to the mesotympanum, eustachian tube and, in particular, the sinus tympani. The creation of a wide extended facial recess with removal of the buttress at the fossa incudis and removal of the incus and head of the maleus will facilitate inspection of the middle ear. Additional techniques are necessary to view the sinus tympani. The fallopian bridge technique, and the infratympanic extended facial recess technique may allow better visualization of the middle ear. CONCLUSION: Mastoidoscopy offers a safe alternative to traditional techniques for second-look surgery. The morbidity appears similar to traditional techniques.

Cholesteatoma, Middle Ear↗

Ossiculoplasty in young children with the Applebaum incudostapedial joint prosthesis.

OBJECTIVE: To evaluate the performance of the Applebaum incudostapedial joint prosthesis in young children in terms of hearing results and long-term stability despite continuing eustachian tube dysfunction and otitis media. STUDY DESIGN: Retrospective review of all Applebaum prostheses placed in children at our institution from June 1993 to June 1998. RESULTS: In 1993 Applebaum proposed the use of a hydroxylapatite ossicular prosthesis as an alternative to incus interposition for the repair of incudostapedial discontinuity. We have used this prosthesis exclusively for the repair of such defects in children over the past 5 years. Among 12 operated ears, all healed, all prostheses remain in place (average duration, 2.6 y), and all children have excellent hearing (mean air-bone gap, 15 dB; range, 5-25 dB). CONCLUSIONS: The Applebaum incudostapedial joint prosthesis restores conductive hearing even in young children. It has been stable in the face of recurrent otitis media and has not interfered with revision surgery. Placement of the prosthesis at primary cholesteatoma surgery should be considered in children.

Adolescent↗

Chronic otitis media sequelae in skeletal material from medieval Denmark.

OBJECTIVES: Chronic otitis media sequelae (COMS) have been identified in archaeological skeletal materials from various ages. COMS reflecting episodes of upper respiratory tract infection may be used as a paleopathological indicator of general health. Estimation of the frequency of COMS may be useful in the gross evaluation of general standard of living. MATERIALS AND METHODS: Temporal bones and auditory ossicles from 659 individuals from two Danish medieval rural parish cemeteries, dated to 1050-1200 and 1150-1350, respectively, were examined otomicroscopically. RESULTS: Osseous fistulae from mastoid abscesses, remodelling of the hypotympanon, and erosion of the incus were among the convincing indications of COMS. A minimum frequency of COMS of 1% to 7% was found. The youngest material displayed the highest frequency of pathological changes. CONCLUSION: Indications of a rising incidence of infectious middle ear disease in early medieval Denmark were found. This may reflect a deterioration of living conditions from the 11th through the 14th centuries.

Bone Diseases↗

Evaluation of eardrum laser doppler interferometry as a diagnostic tool.

OBJECTIVES: Laser Doppler interferometry (LDI) of the eardrum allows noncontact optical analysis of its vibrations in response to sound. Although LDI has been widely used in research, it has not yet been introduced into clinical practice as an adjunctive test for otological workup. The aim of this study was to evaluate LDI as a diagnostic tool in the clinical sphere. STUDY DESIGN: Prospective. METHODS: A measurement system was developed based on a commercially available scanning He-Ne laser Doppler interferometer. The study included 129 eardrums of 79 subjects that were divided into 3 groups: 1) normal subjects and 2) patients with sensorineural and 3) conductive hearing loss (HL). All the patients suffering from conductive HL underwent ossiculoplasty, which allowed confirmation of the final diagnosis, and patients were assigned accordingly to the subgroups malleus fixation, incus luxation, and stapes fixation. RESULTS: The modified LDI system allowed bilateral evaluation of a subject within 30 minutes. No significant difference between normal subjects and patients having sensorineural HL were found. However, it was possible to distinguish between normal subjects and patients with conductive HL. Furthermore, the system had the ability to differentiate between various middle ear diseases. These groups differed statistically significantly in terms of manubrium vibration amplitude and resonance frequency. In malleus fixation significant differences in tympanic membrane movement patterns were found. CONCLUSIONS: Our LDI is applicable in clinical otological practice and serves as a valuable addition to the routine audiological investigations for preoperative evaluation of the mobility and integrity of the ossicular chain.

Adult↗

Long-term stapedectomy results with the McGee stapes prosthesis.

OBJECTIVE: To report long-term follow up on patients undergoing stapedectomy with the McGee stapes prosthesis. STUDY DESIGN: A retrospective case series. METHODS: Charts recording 170 stapedectomies (120 primary and 50 revision stapedectomies) from 1989 to 1999 were analyzed, and audiometric data and the findings at repeat exploration reported. RESULTS: Over a 10-year period, 11.2% of patients had failure of their initial air-bone gap closure with the McGee piston. Seventy-seven percent (in 10 of 13 patients) of McGee pistons that failed were found to have the platinum ribbon displaced laterally from the incus and the piston pushed out of the stapedotomy. The failure rate was not significantly different (P =.72) from that in our patients who underwent placement of a Robinson cup prosthesis (9.5%), but the pattern of displacement was unique. Failures occurred earlier with the McGee piston (average time to failure of 2.5 y) than with the Robinson cup (average time to failure of 8.6 y). CONCLUSION: The McGee piston was found to have a unique pattern of dislocation that is likely related to the malleable nature of the platinum ribbon and loosening of the crimped "shepherd's crook" over time.

Follow-Up Studies↗

Reconstruction of the entire ossicular conduction mechanism.

OBJECTIVES/HYPOTHESIS: Stapes fixation combined with fixation, absence, or malformation of the malleus-incus complex requires an uncommon surgical reconstruction and offers a unique combination of challenges and hazards. This situation may occur in the presence of severe tympanosclerosis, otosclerosis, congenital ossicular malformations, and revision surgery for either stapedectomy or chronic ear disease. In previous reports, this procedure has been grouped with total ossicular reconstruction without much distinction. However, the challenges unique to this problem deserve special consideration. The present report offers a treatment plan for a group of patients requiring reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate. STUDY DESIGN: Retrospective review. METHODS: Three thousand three hundred fifty (3350) charts of patients requiring total ossicular replacement prostheses (TORPs) were reviewed. Of this group of patients, only 21 of 3350 patients from 1977 to 1999 required TORP placement and removal of the stapes footplate. The patients were followed for an average period of 50 months. RESULTS: Hearing results indicated an overall improvement in the air-bone gap of 10 dB, with 52% achieving an air-bone gap of less than 20 dB. Of the 21 cases, 5 revision surgeries were performed. Three were performed because of a displaced TORP (14.2%). and 2 were performed because of extruded TORPs (9.5%). CONCLUSIONS: Reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate can be successfully achieved with improvement of the air-bone gap of less than 20 dB. Hearing results and extrusion rates are comparable to reported results of TORP placement on a mobile footplate. Successful stapedectomy and simultaneous ossicular chain reconstruction can be performed as a single or staged procedure. Special attention is paid to avoid intrusion of the prosthesis into the vestibule.

Adolescent↗

Revision stapes surgery: the malleus to oval window wire-piston technique.

OBJECTIVE: To determine the effectiveness of the malleus to oval window wire-piston revision stapes surgery technique. STUDY DESIGN: A retrospective analysis of 243 stapes procedures performed by the senior author over a 10-year period identified 15 revisions. Five patients underwent a malleus to oval window wire-piston technique. All patients were followed for at least 6 months. The surgical outcome including audiologic data and complications are noted. METHODS: Stapes surgery was performed on an ambulatory basis by way of a transcanal approach under local anesthesia with monitored sedation by the same surgeon using a laser technique and a stapes wire-piston prosthesis. RESULTS: Among revision stapes procedures, there was no significant difference in the air-bone gap closure or complication rate between the incus to oval window and the malleus to oval window techniques. The average preoperative air-bone gap in all revisions was 32 dB, whereas the mean postoperative gap was 10 dB hearing loss. CONCLUSIONS: In experienced hands, revision stapes surgery using the malleus to oval window stapes wire-piston prosthesis is safe and effective.

Adult↗

Optimum tension for partial ossicular replacement prosthesis reconstruction in the human middle ear.

OBJECTIVE: Hearing results from ossiculoplasty are unpredictable. There are many potentially modifiable parameters. One parameter that has not been adequately investigated in the past is the effect of tension on the mechanical functioning of the prosthesis. Our goal was to investigate this parameter further, with the hypothesis that the mechanical functioning of partial ossicular replacement prostheses (PORP) from the stapes head to the eardrum will be affected by the tension that they are placed under. METHODS: Fresh temporal bones were used to reconstruct a missing incus defect with a PORP-type prosthesis. Three different lengths of PORP were used, and the stapes vibrations were measured with a laser Doppler vibrometer using a calibrated standard sound in the ear canal. Eight temporal bones were used. RESULTS: Tension had a very significant effect on stapes vibration. In general, loose prostheses resulted in the best overall vibration transmission. The effects were most marked at the lower frequencies. There was a slight advantage to tight prostheses in the higher frequencies, but much less than the decrement in lower frequencies with tight prostheses. CONCLUSION: In ossicular reconstruction, best stapes vibration results in our model are achieved by shorter prostheses, which result in lower tension.

Cadaver↗

Endoscopic management of limited attic cholesteatoma.

OBJECTIVES: Microscopic postauricular tympanomastoidectomy provides a limited exposure to the attic, especially anteriorly. In contrast, the endoscope offers wide transcanal access to the attic, allowing for complete removal of limited attic disease, possibly without interrupting the ossicular chain. This report evaluates 8 years of experience with transcanal endoscopic management of limited attic cholesteatoma. STUDY DESIGN: Case series. METHODS: Seventy-three ears with limited attic cholesteatoma underwent endoscopic transcanal tympanotomy and extended atticotomy to access and completely remove the sac. Disease was dissected off the tegmen, the medial and lateral attic walls, and the ossicles. Appropriate ossicular reconstruction was performed. The defect was reconstructed with a composite tragal graft. RESULTS: A transcanal endoscopic approach was adequate for removal of disease in all cases. There were no iatrogenic facial nerve injuries. Bone thresholds were stable. Disease was dissected off the head of the malleus and the body of incus with preservation of both in 24 ears. Mean follow-up was 43 months. Five ears required revision for recurrent disease, and eight were revised for failed ossicular reconstruction or persistent perforation. Moderate to severe retraction in other areas of the tympanic membrane was evident in 28 cases; none of these required further intervention. CONCLUSION: An endoscopic technique allows transcanal, minimally invasive, eradication of limited attic cholesteatoma. Preservation of the ossicles coupled with complete removal of disease is more likely with the endoscope.

Adolescent↗

Unexplained conductive hearing loss.

We report a series of unusual clinical cases which exhibit what we have referred to as unexplained conductive hearing loss. Audiometrically, these cases typically display mild to moderate conductive or mixed-type hearing loss and good word discrimination (80% to 100%). The otologic evaluations also lend support to the audiometric findings. When polytome roentgenograms were obtained thr results showed a normal inner ear system. Most often, the otologists' clinical impression was a fixed malleus or congenital stapes fixation, and exploratory tympanotomy with inspection of the ossicular chain was recommended. In each case, the ossicular chain was found to be intact. When the incudostapedial joint was separated the malleus and incus were seen to be mobile. Further, palpation of the stapes resulted in good round window reflexes. Possible explanations for this phenomenon are considered.

Adult↗

Prospective study of resident-performed stapedectomy.

Because of the diminishing number of patients diagnosed with otosclerosis, the adequacy of residency training in stapedectomy techniques remains controversial. A prospective study, conducted from 1986 to 1991, assessed whether or not surgical outcome obtained by residents, under close supervision by otologic faculty, could be improved using a single stapedectomy technique. Comparison of pre- and postoperative hearing results (pure-tone averages of 500, 1000, and 2000 Hz) from 49 cases revealed closure of the air-bone gap to within 10 dB in 68 percent of procedures. Complications included failure to improve the conductive hearing loss, tympanic membrane perforations, transient facial nerve weakness, subluxation of the incus, and adhesions. The failure to improve the success rate in resident-performed procedures relates to the individual learning curve and the limited number of training cases.

Audiometry, Pure-Tone↗

The nucleus double array cochlear implant: a new concept for the obliterated cochlea.

OBJECTIVE: To increase the number of intracochlear electrodes that may be inserted into a totally obliterated cochlea, a special implant has been developed in collaboration with Cochlear Limited. This implant features two separate electrode carriers containing 11 and 10 active electrodes, respectively, as well as a reference electrode located on the receiver-stimulator package. The potential stimulation modes available with this device therefore include monopolar and bipolar stimulation, and stimulation between both arrays. SURGICAL TECHNIQUE: A cochleostomy anterior to the round window provides access to the basal turn (both the scala tympani and the scala vestibuli), and new built connective tissue and bone can be removed until the anterior wall of the basal turn is approached. A second cochleostomy is performed at the second turn caudal of the cochleariform process and 2 mm anterior of the oval window after removal of the incus. New tissue should also be removed if necessary. The two electrode carriers are then placed into the scala tympani of the basal and the scala vestibuli of the second turn, respectively. The remaining surgical procedure is identical with that used for cochlear implantation in patients without obliterated cochleas. PATIENTS: In this clinical study, 10 patients aged 32 to 66 years with an obliterated cochlea each received a double array cochlear implant. All patients had total obliteration of the basal turn either on preoperative imaging or during surgery. Intraoperatively, the second turn was not obliterated in only 4 of 10 patients. Postoperatively, a standard audiologic test battery was used to determine auditory improvement over time. POSTOPERATIVE RESULTS: All patients achieved significantly improved speech understanding when the additional apical electrode array was used, compared with the use of each electrode array independently. No complications occurred. CONCLUSION: In patients with a totally obliterated cochlea, the number of intracochlear electrodes can be increased by use of the Nucleus double array implant. As a result, patients achieve significantly better auditory results.

Adult↗

Choristoma of the middle ear: a component of a new syndrome?

CASE REPORT: Salivary choristoma of the middle ear is a rare entity. The authors report the 26th known case, which is unique in several respects: the patient had abnormalities of the first and second branchial arches, as well as the otic capsule and facial nerve in ways not yet reported. Our patient presented with bilateral preauricular pits, conchal bands, an ipsilateral facial palsy, and bilateral Mondini-type deformities. A review of the literature revealed salivary choristomas of the middle ear to be frequently associated with branchial arch abnormalities, most commonly the second, as well as abnormalities of the facial nerve. REVIEW OF THE LITERATURE: All 25 cases were reviewed and the results reported with respect to clinical presentation, associated abnormalities, operative findings, and hearing results. It has been proposed that choristoma of the middle ear may represent a component of a syndrome along with unilateral hearing loss, abnormalities of the incus and/or stapes, and anomalies of the facial nerve. CONCLUSION: Eighty-six percent of the reported patients with choristoma have three or four of the four criteria listed to designate middle ear salivary choristoma as part of a syndrome. In the remaining four patients, all of the structures were not assessed.

Child↗

Sensory auricular branch of the facial nerve.

GOAL: To better describe the anatomy of the sensory auricular branch of the facial nerve. BACKGROUND: Clinical experience and the medical literature suggest that the facial nerve supplies sensory fibers to the external auditory canal and pinna. The anatomic distribution of these fibers remains poorly defined. METHODS: Ten cadaveric temporal bone dissections with photographic documentation, two clinical cases, and histologic examination of a candidate nerve fiber were collected. RESULTS: The anatomic distribution and histologic confirmation of a facial nerve branch coursing through the posterior wall of the external auditory canal is described. Mean (+/-SD) measurements along the mastoid segment of the facial nerve from the short process of the incus and chorda tympani nerve origin to the auricular branch origin were 11.6 +/- 1.4 mm (range 9-13 mm) and 3.9 +/- 3.0 mm (range 0-8 mm), respectively. Sacrifice of this nerve in a patient resulted in posterior external auditory canal and inferior conchal bowl hypesthesia. CONCLUSION: The anatomy of a facial nerve branch coursing through the external auditory canal is presented. The anatomic and functional findings of this study suggest that this nerve represents an auricular sensory branch. Understanding these anatomic details may help in identifying the main trunk of the facial nerve in surgery, preventing postoperative external auditory canal hypesthesia, as well as understanding the significance of Ramsay-Hunt Syndrome and Hitselberger's Sign.

Adult↗

Cartilage palisade tympanoplasty in sinus and tensa retraction cholesteatoma.

OBJECTIVE: The aim of this study was to investigate whether reconstruction of the eardrum with palisade cartilage technique could prevent retraction of the new eardrum after surgery for sinus and tensa retraction cholesteatoma in children and to investigate the postoperative hearing. STUDY DESIGN: In 32 children aged 5 to 15 years, operated on from June 1995 to October 2000 for cholesteatoma (21 with sinus cholesteatoma and 11 with tensa retraction cholesteatoma) the eardrum was reconstructed with the palisade cartilage technique. Postoperatively, the children were seen as outpatients and were recently reevaluated with otomicroscopy, tympanometry, and audiometry. All patients (100%) were reevaluated on an average of 37 months (range 3-63 months). MAIN OUTCOME MEASURES: Postoperative retractions, perforations, cholesteatoma recurrence, and hearing. RESULTS: At the final examination, posterosuperior retraction was observed in two patients, both operated on for sinus cholesteatoma with reconstruction of the ossicular chain. In those cases, the palisades were not placed in the posterosuperior drilling defect behind the interpositioned incus. All the patients had an intact tympanic membrane at the final follow-up visit. One small perforation was surgically closed during the observation period. No patients developed cholesteatoma during the follow-up period. The postoperative hearing was good, and the hearing did not deteriorate with increasing observation time. CONCLUSIONS: The palisade technique effectively prevented postoperative retraction of the eardrum. The postoperative hearing was good.

Acoustic Impedance Tests↗

Revision stapedectomy in Israel.

OBJECTIVE: To evaluate the audiometric results and surgical findings of revision stapedectomy in Israel. STUDY DESIGN: Retrospective. SETTING Tertiary referral center and private otology practice. PATIENTS: Seventy-eight patients over 13 years. RESULTS: A total of 78 patients were evaluated during a mean follow-up time of 1.5 years. Ten patients with far advanced otosclerosis or dizziness were then excluded. Closure of air-bone gap to within 10 dB was achieved in 67.6% of patients. Pure tone average (PTA, 500-4,000 Hz) improvement in hearing was 22.5 dB. Operative findings at time of revision were prosthesis malfunction (42.6%), incus necrosis (22%), incomplete stapedectomy (11.8%), and fistula (l.5%). Sensorineural hearing loss occurred in one patient. The technique of revision involved regrafting the oval window and the use of a piston prosthesis. CONCLUSIONS: The surgical findings and results in this study are similar to those in other large studies. Although success was less than in primary stapedectomy, the results justified revisions. Hearing gains were dependent on surgical findings, the number of previous revisions, and associated ossicular problems.

Adolescent↗