Hearing loss in children has been attributed to infectious diseases since long before. They include congenital infections such as congenital rubella syndrome and congenital syphilis as well as acquired infections such as meningitis, encephalitis, otitis media, measles and scarlet fever. Development and distribution of vaccines (e. g., rubella, measles, Japanese encephalitis, pneumococci, Hib and so on) and antibiotics (e. g., penicillin) have turned many of them to rare diseases and reduced the number of children with hearing loss as the sequelae in developed countries. Taking a look at the situation in developing countries, however, infants with congenital rubella syndrome have been born in countries where rubella-containing vaccine has not been introduced into the national immunization program, and many other infectious diseases have been in the out-of-control condition. Even in Japan, a couple of infectious diseases remain the threat of hearing loss, mainly congenital cytomegalovirus infection and mumps.

Myringitis is the inflammation of the tympanic membrane between the middle and external ear. We reported a case of myringitis that was treated surgically as conservative treatment was ineffective. A 34-year-old woman visited our hospital with a complaint of slight otalgia. She was previously treated, conservatively, by other doctors, but the myringitis persisted. The tympanic membrane was perforated due to repeated irritation from itchiness. Otomicroscopic examination revealed a granulose and slightly rubor, protruded lesion at the posterosuperior quadrant of the tympanic membrane. Surgical treatment was performed in order to resect the neoplastic lesion. The pathological diagnosis established the occurrence of follicular hyperplasia of the tympanic membrane. This lesion had penetrated all layers of the tympanic membrane, and was caused by the tympanic membrane’s repeated irritation. Therefore, the lesion was irreversible. We believe that that this would require surgical treatment. To prevent the recurrence of myringitis, the tympanic membrane should not be repeatedly irritated, and the itchiness should be treated as soon as possible.

Widely denuded bony ear canal presents an important issue during the surgical treatment of severely lateralized tympanic membranes, as it may lead to prolonged epithelialization and disease recurrence. Surgical treatment for a lateralized tympanic membrane was performed in 6 cases. All patients were followed for more than one year. After the reconstruction and appropriate placement of the tympanic membrane, a postauricular periosteal-pericranial flap was applied to the denuded bony canal. Any necessary ossicular reconstruction was then performed. Reepithelization occurred faster in the 6 cases described in this study than in previous cases in which a split-thickness skin graft was applied to the denuded bone. Of the 6 ears, 4 (66.7%) displayed post-operative air-bone gaps of 20 dB or better. In 3 cases, the postoperative findings of the tympanic membrane showed a favorable appearance; anterior blunting was seen in 2 cases. Medial meatal fibrosis occurred in the remaining case. A postoperative computed tomography examination showed the inadequate removal of the anterior bony ear canal in the cases with poor tympanic membranes. Periosteal-pericranial flaps are long enough to cover the entire bony wall and are thin enough to be easily manipulated within the ear canal. Therefore, such flaps are useful for covering the bony ear canal. In addition, they are easy to prepare and produce promising results.

Klippel-Feil syndrome is characterized by a clinical triad consisting of a short neck, decreased head mobility, and low occipital hairline; additionally, the triad is accompanied by hearing loss in 80% of patients. We encountered a patient with this syndrome who had undergone stapedotomy for bilaterally present stapes anomalies. The patient was a 10-year-old boy with a chief complaint of hearing loss in both ears. Pure tone audiometry at the first visit showed combined hearing loss in both ears (76.7 dB in the right ear and 70 dB in the left). Exploratory tympanotomy was performed in his right ear. Shortened incus long process, missing stapes, oval window atresia, and aberrant facial nerve were observed. Stapedotomy-M was performed, which improved his hearing; however, postoperatively, transient facial nerve paralysis was observed. Further, similar ossicular chain anomalies were seen on the contralateral side. Stapedotomy-M on the left side did not result in significant improvement in his hearing. Since various middle-ear anomalies are observed in patients with this syndrome, we should be careful to avoid causing intraoperative injuries during exploratory tympanotomy and tympanoplasty.

The hearing results after surgery for tympanosclerotic stapes fixation have been unsatisfactory. Stapedectomy for tympanosclerotic fixation seems more difficult than that for otosclerosis because most cases have fixation or erosion of the malleus and/or incus. In addition to difficulties associated with the available surgical technology, there is risk of deafness caused by internal otitis (because of poor hygiene of the tympanic cavity), and severe dizziness caused by a perilymphatic fistula and subluxation of the prosthesis into the vestibule.

We present the postoperative hearing results of 52 ears that were affected by tympanosclerotic stapes fixation. Stapes mobilization was performed on 33 ears in three treatment groups: small fenestra stapedectomy (SFS) using interposed 4-mm wire Teflon-piston between the incus and oval window was performed in eight ears; stapedotomy with interposed malleus attachment prosthesis (MAP) between the malleus and oval window in three ears; and stapedectomy with interposed total ossicular replacement prosthesis (TORP) between the tympanic membrane and oval window in eight ears. For ears with tympanosclerotic stapes fixation, we concluded that neither stapes mobilization nor SFS were effective for improving hearing, whereas stapedectomy with ossiculoplasty using TORP was very effective. However, the differences in the postoperative hearing gain and the air-bone gap among these three groups were not statistically significant.

When severe dizziness occurs postoperatively, CT is necessary to investigate the condition of the inner ear. It is essential to carefully evaluate such CT views for the existence and position of an air bubble in the inner ear and the degree of protrusion of the prosthesis into the labyrinth. Thus, CT images may be useful to predict the severity and prognosis of complications after surgery for tympanosclerotic stapes fixation.

Facial synkinesis, a sequela of peripheral facial palsy, has a painful impact on patients. Recent advances in rehabilitation have reduced the incidence of facial synkinesis and tinnitus, which occurs due to abnormal contraction of the stapedius muscle (stapedial tinnitus) following facial palsy. We report a case of chronic otitis media and tinnitus due to abnormal stapedius muscle contraction following Ramsay Hunt syndrome, which resolved after performing dissection of the stapedius muscle tendon. A 70-year-old man presented to our outpatient clinic bilateral hearing loss, otorrhea, and tinnitus on the left side upon eye closure. Otoscopic examination revealed tympanic membrane perforations in both ears. Pure tone audiometry revealed bilateral mixed hearing loss and left low-tone hearing loss upon eye closure. Tympanoplasty and dissection of the stapedius tendon in the left ear and myringoplasty in the right ear were performed. Subsequently, tympanic membrane perforations in both ears were closed, and the bilateral air-bone gap diminished. The left-sided tinnitus and low-tone hearing loss upon eye closure also resolved. The patient was satisfied more with regard to the elimination of tinnitus than with hearing improvement or disappearance of otorrhea. Since stapedial tinnitus following facial palsy can be corrected by surgery, attention should be given to this phenomenon.

Objective: To investigate the erosion of the incus and stapes caused by congenital cholesteatoma (CC).

Method: We retrospectively reviewed 36 cases of CC that underwent surgical treatment at our hospital from 2006 to 2015. We used surgical records, videos, and computed tomography (CT) scans to determine the type, size, and position of the CC, the erosion of the ossicles and ossicular reconstruction method used.

Result: Among the reviewed cases, the average age at first operation was 8 years, and 4 patients were over 20 years of age. Six cases were classified as Potsic stage I, 3 as stage II, 24 as stage III, and 3 as stage IV.

In stages III and IV (27 cases), the ossicular chains were affected by CC. Out of these cases, the CC existed both anterior and posterior to the malleus handle in 14 cases, only posteriorly in 11, and originated from areas other than the malleus handle in 2.

The stage III cases showed the most variation with regard to stapes erosion; 2 cases had an intact long process of the incus and erosion of the anterior crus and footplate of the stapes. These cases were treated with type I tympanoplasty (TP). The long process of the incus was eroded in the other 22 stage III cases; among these, 2 cases had an intact stapes and were treated with type III TP while 4 had one eroded crus of the stapes and were treated with type III or IV TP. Further, 5 had lost both crura of the stapes and 11 had only the foot-plate remaining; thus, 16 cases were treated with type IV TP.

Conclusion: We confirmed that the type of ossicular erosion depended on the type, position, and size of the CC.

Congenital cholesteatoma of the middle ear can be classified into two types: the closed type is characterized by the development of a keratotic cyst, and the open type is characterized by a wide, flat section of epidermal tissue that forms part of the middle ear mucosa. The closed type can be easily diagnosed by the observation of a white mass through the tympanic membrane (TM). However, the open type is difficult to diagnose otoscopically. In addition, cholesteatoma can develop as a secondary condition. In some cases of secondary cholesteatoma, the epidermis extends into the medial surface of the TM from the margins of a TM perforation because of the inflammation of the tympanic cavity and the lack of retraction of the pars flaccida of the TM. Secondary cholesteatoma tends to affect the aged and is characterized by large TM perforations, epithelial invasion from around the handle of the malleus, long-lasting perforations, and restricted mastoid air cell growth. We encountered three cases of cholesteatoma with unusual characteristics. The cases involved younger individuals, and exhibited marginal small perforations of the TM; moreover, the causes of the TM perforations were unclear, and the patients’ mastoid cells showed good aeration and growth. At first, these patients were diagnosed with otitis media with effusion (OME), acute otitis media (AOM), or calcification of the TM because they had a white TM, which did not appear perforated. We now suspect that these patients had open-type congenital cholesteatoma with TM perforation. Clinicians should be aware that open-type congenital cholesteatoma at the medial surface of the TM can be misdiagnosed as OME, AOM, or calcification of the TM. In cases involving TM perforations, differentiating between open-type congenital cholesteatoma and secondary cholesteatoma is important.

Endaural type-I tympanoplasty, using an ear speculum, is the most commonly performed minimally invasive surgery for the treatment of chronic otitis media at our center. In this approach, a minimum incision of the meatal skin is made in the bony portion of the ear canal. As the surgical field is narrow in this procedure compared to the retroauricular approach, larger grafts can often limit the view of anterior part of the eardrum, specifically in cases with curved ear canal or total perforation of the eardrum. In such cases, to enable better surgical vision, small, multiple pieces of graft are useful to repair the perforation. Single and multiple graft piece tymphanoplasties for perforations of various sizes were compared for postoperative hearing restoration and closure rates. For larger perforations, multiple piece grafting showed significantly higher closure rate than single piece grafting. However, in cases of medium to small sized perforations there was no significant difference between single and multiple piece grafting in terms of perforation closure rate or postoperative hearing restoration. Therefore, we conclude that multiple-piece grafting, through an ear speculum, enhances the efficacy of type-I tympanoplasty in repairing large perforations.

Malignant tumors of the temporal bone are rarely seen in the daily practice of otology. Although there is a lack of consensus as to the most appropriate treatment, the most frequently reported treatment is surgical resection. As a pathologically tumor-free margin could improve survival, the establishment of surgical techniques is extremely important in the management of carcinoma of the temporal bone. Here, we present two representative cases of carcinoma of the temporal bone; one was squamous cell carcinoma of the external auditory canal (stage T2N0M0) that was treated using lateral temporal bone resection, and the other was squamous cell carcinoma of the middle ear (stage T4N0M0) that was treated using subtotal temporal bone resection. Surgical techniques and preoperative planning of the surgeries based on the images are discussed.