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Biomedical subjects

D Portmann

Publications and source records attributed to D Portmann.

At least 37 records · Page 2Linked to original sources
PubMed1997

[ORL and speech aspects in DiGeorge syndrome].

The DiGeorge syndrome presents clinically as a combination of a congenital cardiopathy with immune deficiency and predisposition to infections, signs of hypoparathyroidis with severe hypocalcaemia in the neonatal period, and facial dysmorphism. New techniques in molecular cytogenetics (in-situ fluorescent hybridisation--FISH) have provided evidence of microdeletion of chromosome 22q11 in most cases of the DiGeorge syndrome. There is an important overlap between this syndrome, the velo-cardio-facial syndrome, and certain other cono-truncal cardiac anomalies which are linked with the same microdeletion syndrome. Basing their observation on a case of the partial syndrome, the authors emphasise the otological and maxillo-facial aspects, and especially the effects on speech and language. It is essential to carry out repeated audiometric testing to exclude an audiometric cause for the speech and language problems. At the same time, thorough speech and language assessment is necessary to establish the degree of velar insufficiency (rhinolalia). These will guide the speech therapy rehabilitation, and quantify the psycho-affective component. Surgery on the palate may be a possibility, depending on the progress in speech and language improvement.

DiGeorge Syndrome↗
PubMed1997

[Cholesteatoma of the middle ear in children. Clinical, developing and therapeutic study in a series of 215 consecutive cases].

A retrospective study of 215 cases of cholesteatoma in 199 children operated on from 1985 to 1996 was conducted. Mean age of the children was 9.6 years. Bilateral cholesteatoma was present in 32 cases (8%). The cause was congenital in 32 cases (16%), iatrogenic in 24 cases (11%) and secondary or acquired in 63%. The surgical procedure was a closed technique in 88%, an open technique with or without ossiculoplasty in 10%, and exclusion of the ear using the Rambo technique in exceptional cases (1%). The facial nerve was exposed in 17%. A labyrinth fistula was discovered in 4%, and an intracranial suppuration in only 0.5%. A secondary open procedure was required in 5 cases (2.5%) and secondary exclusion 3 times (1.5%). The ossicular chain was interrupted in 24.2% of the cases and ossicular rehabilitation was performed in 68% of the cases (39% type II, 29% type III), including 49% during the first procedure. At least 2 operations were needed in 62% of the children and 21% had 3 operations. Residual cholesteatomas were observed in 21.5% of all children in the series, 33% in those with a second operation. Recurrence rate was 10%. Mean hearing loss was 28 dB for a mean gain of 4 dB. Post-operative hearing capacity was socially useful in 64% of the cases, including 12% who had normal hearing. Labyrinthization was observed in 15 cases, including 4 cases with total hearing loss. These result are in general agreement with data in the literature. Our strategy is based on a closed procedure for cholesteatomy and systematic re-evaluation at 1 year. The open technique is useful for very extensive lesion on a narrow mastoid, either as second intention procedure or more rarely as a first intention procedure. Management in children differs from that in adults since in adults the destruction has usually progressed further in a mastoid which accepts an open procedure more readily.

Adolescent↗
PubMed1996

[ORL involvement and Noonan syndrome].

The authors reports on the case of a young, 17-year-old girl, suffering from Noonan's syndrome, with a bilateral conductive hypoacusia. Surgical exploration of the tympanic cavity revealed on one side an attical fixing of the ossicular chain and on the other side a hyperlaxity of the incudostapedial joint. On the basis of this observation, they stress the neglect of a study of the hearing of such patients, whose verbal expression and intellectual level are also impaired. Finally, they study the type of deafness involved and its etiopathology, suggesting the hypothesis of an anomaly in the development of the first branchial arch.

Adolescent↗
PubMed1995

[The use of osseointegration of hearing aids].

The principles of osseointegration defined by Branemark are reviewed. The authors describe the otological applications of titanium implants: bone anchored hearing aids (BAHA). The good results obtained depend on the selection of the patients and the observance of a rigorous surgical technique. The present paper summarizes our results with 32 BAHA implanted in 27 patients.

Adolescent↗
PubMed1995

[Bone anchored hearing aid: the Bordeaux experience].

Bone anchored hearing aids (BAHA) based on the concept of osseointegration offer the possibility of restoring hearing in cases of conduction or mixed deafness. The authors report on their experience with 42 implants in 35 patients, dived into two major groups, with bilateral agenesia on the one hand (15 cases) and problems of chronic otitis media (20 cases) on the other hand. The functional results are excellent, with 30 patients fitted with 32 HC200 or HC300 prostheses. The authors stress the necessity of strictly observing the audiometric criteria and the determinant factors of osseointegration, essentially the surgical technique.

Adolescent↗
PubMed1993

Concerning the late diagnosis of certain cerebellopontine angle tumors.

The sensitivity of diagnostic methods for cerebellopontine angle lesions (auditory nerve electrophysiology, computed tomography scanning, magnetic resonance imaging) has increased in recent years. Paradoxically, in our experience this has not led to an increased percentage of tumors detected at an early stage. In 1975 20% of tumors were confined to the internal auditory canal, whereas only 6.6% were intercanalicular in 1988. A detailed analysis of 120 recent cases included first symptoms, time between first symptoms and diagnosis, symptoms at diagnosis, and size of tumor. We conclude the low percentage of intracanalicular tumors in the recent series results from: (1) rapid growth in some cases; (2) slow enlargement of the tumor over a long time without compression of adjacent cranial nerves leading to a paucity of symptoms; (3) misleading clinical pictures, including sudden hearing loss and hydrops; and (4) tumor symptoms masked by unrelated pathologic processes (otosclerosis, presbyacusis, trauma).

Journal Article↗
PubMed1993

[Benign adenoma of the middle ear].

Benign adenoma of the middle ear is a tumor from glandular mucosa. In most of the cases, it is revealed between 40 and 60 year old, by a conductive hearing loss with a greyish mass behind intact tympanic membrane. Scanography demonstrated a no specific tumor, without bone destruction. The ossicular chain is usually respected. The histologics findings show two distinctive type of cells: glandular and neuroendocrine differentiation. The treatment is surgical. A regularly follow up is necessary to diagnostic recurrence.

Adenoma↗
PubMed1993

[Widened retrolabyrinthine approach: application to the surgery of acoustic neuroma].

For a long time, the role of the widened retrolabyrinthine approach (WRLA) in surgery of the posterior fossa was restricted to functional surgery of the erebellopontine angle (CPA). The widening technique described makes it possible to adapt it to surgery for the exeresis of APC tumors, irrespective of their histological nature. We have used this approach to treat 19 neurinomas of the VIII and 11 other lesions. The results obtained in the preservation of facial function and hearing match those recorded with more traditional approaches, such as the translabyrinthine and suboccipital retrosigmoid approaches. By its modularity in the access space, it is the multi-purpose approach for the CPA. In our therapeutic armamentorium, given its possibilities to preserve hearing, it has taken the place of the less flexible and more dangerous suboccipital approach.

Ear, Inner↗
PubMed1993

[Transfrontonasal approach: development of a mixed approach to ethmoid tumors. Experience of 12 cases].

Our experience in approaching ethmoid tumors through a combined pathway is currently based on 81 patients. 69 of them were malignant tumors treated by the neurosurgical subfrontal and paralateronasal transfacial rhinological approach. For the past two years, we have adopted the technical modification described in Bordeaux by Pinsolle and San-Galli enabling the approach of the ethmoid bone by means of a unique (Unterberger's scalp) incision and a unical frontonasal flap. 12 patients benefitted from this transfrontofacial access. The advantages and disadvantages of the technique are discussed.

Ethmoid Bone↗
PubMed1992

[Current data on barotraumatic ear disease with isolated vestibular expression during apnea diving].

The authors have taken an interest in isolated vertiginous symptoms observed from free diving patients. In order to achieve a current bibliographic synthesis, they resume, on basis of 6 cases, the basic principles of the physics theories and the etiopathogenic mechanisms. They try so to help in the understanding of the vestibular clinical signs observed in this successful sport.

Acoustic Impedance Tests↗
PubMed1992

[Apropos of the infratemporal pre-auricular approach (its value in the approach to the midline skull base].

The role of the infratemporal pre-auricular pathway to the median base of the skull resides in the fact that it enables very wide access to this region while sparing the facial and auditory nerves. Among the operations performed on the approaches of the base of the skull by their teams, the authors chose a particularly typical observation dealing with the indication of this pathway: a 34-year old male with a giant cholesteatoma of the tip of the petrous bone totally surrounding the internal carotid artery along its entire (intrapetrous) length, from the cervical region to the cavernous sinus, having invaded the clivus and pushed back the brainstem. This lesion is located on the side of the only healthy ear. The patient is completely deaf in the ear on the other side. The operation enabled its complete exeresis while respecting the facial, auditive and vestibular functions.

Adult↗
PubMed1992

[AIDS and lymphoma of the ear].

The authors describe the circumstances for detection of an AIDS lymphomatous complication revealed by an otological picture in one patient. The lymphomatous pathology not related to HIV is reviewed. The particularity of these lymphomas, when they are related to HIV, is their frequent initial extra-ganglionic location, their high grade histological type and their poor prognosis. The authors emphasize the need to perform a biopsy rapidly when confronted with any external otitis resistant to general and local treatments.

Adult↗
PubMed1992

[Osseointegrated implants in facial prosthesis and hearing aids].

The principles of osseointegration defined by Branemark are reviewed. The authors describe the otological applications of titanium implants: bone anchorage hearing aids (B.A.H.A.) and ear epitheses. Obtaining good results depends on the selection of the patients and the observance of a rigorous technique.

Ear, External↗
PubMed1991

[Post-traumatic facial paralysis without any fracture of the petrous bone].

Post-traumatic facial palsies are generally observed after a transverse, longitudinal or comminuted fracture of the pars petrosa. Occasionally, the fracture does not involve the Fallopian aqueduct and another mechanism must be sought. The authors present a case of facial palsy that occurred after a Le Fort's fracture of the base of the skull, not involving the Fallopian aqueduct on the CT scan. This was confirmed by surgical exploration. The authors recall the possibility of a facial palsy through the stretching of the nerve by the petrosal nerve.

Adult↗