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Audiologic evaluation of hearing-impaired infants and children.

With the advent of auditory brainstem response (ABR) and otoacoustic emission (OAE) testing, as well as the widespread use of universal hearing screening programs, the audiologic assessment of hearing-impaired infants and young children has become more frequent in recent years. When assessing a hearing-impaired infant, it is necessary to obtain as much frequency and ear specific information as possible prior to selection of hearing amplification. An overview of the battery of tests and the need for a diagnostic center that is well versed in both physiologic and behavioral tests will be addressed.

Audiometry↗

Osteopetrosis: a scanning electron microscopic study.

Scanning electron microscopic investigation was conducted with bone in a case of osteopetrosis by comparing it with normal cortical bone. The fractured surface of the osteopetrotic bone was devoid of the orderly constructed lamellar haversian system seen in normal bone. It was occupied by a massive smooth cartilaginous matrix containing scattered rough areas of abnormal ossification. Tortuous channels were randomly distributed and corresponded to the abortive marrow spaces. The presence of many irregular fracture lines reflected the fragility of the bone. The findings support the concept of defective remodeling in the pathogenesis of osteopetrosis and provide a further explanation for the abnormal fragility of bone in osteopetrosis.

Adult↗

The presence of a widened vestibular aqueduct and progressive sensorineural hearing loss in the branchio-oto-renal syndrome. A family study.

OBJECTIVE: A new large family with the BOR syndrome is reported with special reference to the presence of a widened vestibular aqueduct and a progressive sensorineural component in the mixed hearing loss. A review of the BOR literature of 184 patients is given. SETTING: University Hospitals. RESULTS: A BOR family with 17 affected members was studied. Fourteen of those 17 were still alive and 12 of those cooperated in this clinical study. Detailed radiological studies showed in three out of 12 affected family members a widened vestibular aqueduct and progressive sensorineural hearing loss. This raises the question whether there is a true correlation or whether those are coincidental. CONCLUSION: In our family with the Branchio-Oto-Renal syndrome, a widened vestibular aqueduct and progressive hearing loss is found in a few affected family members. Imaging of the temporal bones and long-term audiometric follow-up could help to reveal whether the widened vestibular aqueduct is the cause for the progressive hearing loss.

Adolescent↗

Tympanic membrane reconstruction using formaldehyde-formed autogenous temporalis fascia: twenty years' experience.

This study describes 20 years' experience with autogenous temporalis fascia that is formed and shaped by formaldehyde cross-linking with special Fasciaform (Hear America, Palo Alto, Calif.) molds in the repair of large tympanic membrane perforations. One hundred twenty operations in 113 patients were performed between 1973 and 1993 to close large perforations in patients with intact ossicular chains. All perforations were successfully closed by this technique. Audiometric studies indicated that the postoperative air-borne gap was closed to within 0 to 10 dB in 63% and to within 0 to 20 dB in 97%. One patient had a 15-dB sensorineural hearing impairment. Graft lateralization requiring revision occurred in three patients, two of whom had previous unsuccessful tympanoplasties. Comparisons of adult vs. pediatric groups and primary vs. revision groups were made. The technique ensures the removal of any ectopic epithelium on the medial surface of the tympanic membrane remnant and provides for easy graft placement and stability during healing without the use of middle ear Gelfoam (Upjohn Co., Kalamazoo, Mich.). The formaldehyde-formed fascia graft or Fasciaform graft technique provides an effective method of closing large perforations with excellent functional results and minimal complications.

Adolescent↗

Results of tympanoplasty with titanium prostheses.

To improve the hearing results associated with tympanoplasty, we performed the operation in 113 cases (124 ears) using ossicular replacement prostheses made of titanium. The results demonstrated that among all the operated ears, 115 of 124 (92.7%) had a hearing gain of more than 10 dB. The average improvement of pure-tone hearing was 24.6 dB, and the average reduction of the air-bone gap was 23.2 dB. No rejection reaction or recurrent cholesteatoma was found. This operative technique, based on animal experiments and applied anatomic study of the suprastapedial structure in human beings, has had a good rate of success and has resulted in remarkable hearing improvement of a relatively large magnitude. The application of a new material-titanium-which has high biocompatibility with human body tissues, is in large measure the reason for this success. The ossicular replacement prosthesis was appropriately designed and manufactured. It fit well, could be tightly joined to the suprastapedial structure, and was difficult to displace. We also adopted the method of enlarging the tympanic isthmus, which led to good aeration of the mastoid and attic, resulting in the best state of mobility of the tympanic membrane. All these factors contributed to the effective hearing gain.

Adolescent↗

Surgical modification of the difficult mastoid cavity.

This retrospective study used a computerized otologic database to identify patients undergoing revision surgery for a draining cavity unresponsive to medical therapy, with at least a 2-year follow-up. The surgical intervention involved partial mastoid obliteration and restoration of the middle ear space by use of cartilage reconstruction of the tympanic membrane. Ossicular reconstruction was achieved with either a partial or total ossicular replacement prosthesis. Because this technique involved contouring the mastoid cavity, the problems that usually occur, such as drainage or debris collection, were alleviated. In addition, re-establishment of the middle ear space often restored hearing. A completely dry cavity was achieved in 18 of 20 patients. An overall statistically significant improvement in hearing (P < 0.05) was obtained, with the mean pure-tone average air-bone gap decreasing to 16.1 dB from 36.5 dB. This technique has proven to be a useful adjunct in the surgical management of the chronically draining cavity.

Adolescent↗

Malleus-to-footplate prosthetic interposition: experience with 265 patients.

Absence of the long process of the incus with or without absence of the stapes head accounts for more than 80% of ossicular discontinuities. Total or partial replacement prostheses, made of various materials, are interposed to restore the transfer function of the middle ear. To simplify ossicular reconstruction, reduce operative times and costs, improve functional outcomes, and avoid the risk of infections, we have adopted, during the past 10 years, a technique that makes use of a personally designed alloplastic prosthetic device. The prosthesis connects the malleus to the footplate, even in the presence of the stapes superstructure. This malleus-to-footplate prosthesis consists in a plastipore-coated steel piston and hydroxyapatite head, complete with a groove. The groove is placed beneath the malleus neck after dissection of the tensor tympani tendon and the shaft of the piston on the footplate. Two hundred ninety primary ossiculoplasties with the malleus-to-footplate prostheses were performed in 265 patients from 1986 to 1995 in the ENT Department of the University of Verona. The average postoperative air-bone gap at 0.5 to 3 kHz was 11 dB at 1 year and 14 dB at 5 years. These outcomes are significantly better than those personally obtained previously with ossicular or alloplastic prostheses. No extrusions occurred. The structural characteristics of the malleus-to-foot-plate prosthesis endow the prosthesis with a high degree of biocompatibility and stability and optimal sound-transfer function. The rationale for this particular ossiculoplasty procedure is discussed.

Adolescent↗

Ossiculoplasty with polymaleinate ionomeric prosthesis.

With the continued concern over the possible transmission of viral infections through homologous middle ear implants, there is increasing pressure to develop a truly biocompatible alloplastic middle ear prosthesis. The polymaleinate ionomer, which has been used in dentistry as a filling and luting material for more than 15 years, has recently been used to construct total and partial ossicular replacement prostheses. In an attempt to evaluate these new implants, a multicenter prospective clinical trial was initiated. To date, 92 patients have undergone implantation. The follow-up interval ranged from 3 months to 22 months. Although it is premature to discuss the long-term results, the preliminary surgical experience and audiometric data with these implants are reviewed. From a surgical perspective, the ionomeric prostheses were easily contoured with a diamond burr and were not prone to shattering. Preliminary follow-up audiometric data were available on 80 patients (59 partial ossicular replacement prostheses and 21 total ossicular replacement prostheses). Of the 59 partial ossicular replacement prostheses the air-bone gaps (average of 500 Hz, 1 kHz, 2 kHz and 3 kHz) were as follows: 0 dB to 10 dB, 15 (25%) of 59; 11 dB to 20 dB, 20 (34%) of 59; 21 dB to 30 dB, 11 (19%) of 59; and greater than 30 dB, 13 (22%) of 59. Of the 21 total ossicular replacement prostheses the air-bone gaps were as follows: 0 dB to 10 dB, 6 (29%) of 21; 11 dB to 20 dB, 6 (29%) of 21; 21 dB to 30 dB, 5 (24%) of 21; and greater than 30 dB, 4 (19%) of 21.

Acoustic Stimulation↗

Cavum major tympano-ossiculoplasty.

OBJECTIVE: To evaluate the success of tympano-ossiculoplasty in patients with previous canal wall down mastoidectomy. STUDY DESIGN: A retrospective review of 79 patients who underwent cavum major tympano-ossiculoplasty from a total of 1,910 tympanomastoidectomies from 1976 to 1998. OUTCOME MEASURES: The results of air-bone gap closure, surgical findings, and revision surgery are presented. RESULTS: In 63% of patients, the air-bone gap closed to within 20 dB with a mean gain of 14.7 dB. CONCLUSION: A significant percentage of patients will gain substantial improvement in their hearing after cavum major tympano-ossiculoplasty with minimal risk.

Adolescent↗

Middle ear sound transmission: a field of early interest to Merle Lawrence.

Merle Lawrence, together with his teacher, E.G. Wever, made early contributions to the problem of middle ear sound transmission. Experiments were mostly done in cats and studied the effects of stepwise destruction of the middle ear on cochlear input and the effects of changes in middle ear air pressure on sound transmission. Their experiments provided the first physiologic evidence that the origin of auditory distortion lies in the inner ear, not in the middle ear. Lawrence also demonstrated a dual innervation of the tensor tympani muscle. With the aid of newer instrumentation, the authors studied the effects of alterations of the middle ear cavity configuration, stepwise removal of the middle ear structures, restriction of ossicular mobility, and ossicular mass loading. Our experiments caused partial decoupling of the tympanic membrane and distortion of the axis ligaments, the apparent weak elements of the middle ear system in cats. The placement of the prosthesis along the mallear handle did not affect sound transmission, as long as the connection between malleus and prosthesis was made inflexible.

Animals↗

The otolithic organ as a receptor of vestibular hearing revealed by vestibular-evoked myogenic potentials in patients with inner ear anomalies.

The human vestibule has preserved an ancestral sound sensitivity and it has been suggested that a reflex could originate from this property underlying cervical muscle micro-contractions secondary to strong acoustic stimulation. Previous studies have established that an early component of loud sound-evoked myogenic potentials from the sternocleidomastoid muscle originate in the vestibule. This is based on findings that the response can still be obtained from patients with complete loss of cochlear and vestibular (semi-circular canal) function. Our data confirm, in a more direct way, a saccular origin of this short-latency acoustic response and verifies that a saccular acoustic response persists in the human ear. The contribution of this response to the perception of loud sounds is discussed. It is concluded that vestibular response to sound might be used to assist in the rehabilitation of deafness.

Adolescent↗

Auditory brainstem response in tammar wallaby (Macropus eugenii).

Auditory brainstem responses (ABR) elicited by click and tonal stimuli were recorded from the tammar wallaby (Macropus eugenii), a marsupial mammal. The morphology, threshold, amplitude, and latency of ABRs recorded in the tammar wallaby are similar to those of other marsupials and mammals used in auditory research, including humans. Thresholds determined by an algorithm employing cross-correlation and by conventional visual detection methods were comparable. The findings from this study indicate that tammar wallaby is a suitable model for auditory research and that algorithms employing cross-correlation are useful for detection of the ABR waveform.

Acoustic Stimulation↗

Cholesteatoma otitis media with intact ossicular chain.

Thirty-one cases of cholesteatoma with intact ossicular chain were reviewed to examine the extension of cholesteatoma, operation procedures, pre- and postoperative hearing levels, and postoperative condition of the ear drum. They were operated on at Departments of Hyogo College of Medicine and Osaka University Medical School from 1989-1996, which were 20.4% of all of the primary cholesteatoma cases (n = 152). Twelve cases located at attic were operated by atticotomy or canal wall up and scutumplasty, 17 cases located at attic/antrum were operated by canal wall down and reconstruction, and two cases located around stapes were operated through the ear canal. Preoperative hearing level was mild (34.2 +/- 18.4 dB) and postoperative one was slightly improved (28.7 +/- 11.2 dB). In ossicular reconstruction, modified Wullstein type III method showed better hearing gain than Wullstein type I method. Postoperative attic retraction and/or pocket occurred in 15 cases (48.4%), but no recurrent cholesteatoma was found during the present follow-up period. These findings suggested that cholesteatoma with intact ossicular chain showed good postoperative hearing results, but postoperative retraction of the ear drum should be followed up for a long time.

Adolescent↗

Bilateral same day surgery for bilateral perforated chronic otitis media.

OBJECTIVE: Bilateral same day surgery has been performed rarely because of the risk of postoperative sensorineural hearing loss following conventional myringoplasty or tympanoplasty (CMT). Simple underlay myringoplasty (SUM) through the ear canal has been developed by Yuasa R, Saijo S, Tomioka Y, et al. Office closure of eardrum perforation with fibrin glue (in Japanese), Otolaryngol Head Neck Surg (Tokyo) 1989;61:1117-1122, which has little risk of sensorineural hearing loss. We tried bilateral same day surgery using this technique and evaluated its outcome. METHODS: Of 86 cases with bilateral perforated chronic otitis media that we treated between 1995-1997, 25 cases underwent bilateral same day surgery. Bilateral SUMs was performed on seven patients, SUM and CMT on 16 patients, and bilateral CMTs on two patients. RESULTS: Closure of perforation was successful in 18 patients (72%) on both sides and in seven patients (28%) on one side. Postoperative air-bone gap of less than 20 dB was achieved in 15 cases (60%) on both sides and in 23 cases (92%) on one side. CONCLUSION: Bilateral same day surgery for bilateral perforated chronic otitis media is possible if the operative indications are considered.

Adolescent↗

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